Program Book

XV th
NH City Hotel and Tower
Bolivar 160

International Society of Ocular Oncology Committee
President
Jacob Pe’er, MD
Hadassah, Israel
Secretary
Tero Kivela, MD
Helsinki, Finland
Chaiperson Membership
Michael Giblin, MD
Sydney, Australia
Chairperson-Research
William Harbour, MD
St. Louis, USA
Chairperson-Program
Martine Jager, MD, PhD
Leiden, the Netherlands
Executive Secretary
Sandra Dailey
Philadelphia, USA
Local Organizing Committee
2
Vice-President
Bertil Damato, FRCS
Liverpool, UK
Treasurer
Timothy Murray, MD
Miami, USA
Chairperson-ByLaws
John Hungerford, FRCS
London, UK
Chairperson-Nominated
Committee
Jerry A. Shields, MD
Philadelphia, USA
Chairperson-Website
Shahar Frenkel, MD, PhD
Jerusalem, Israel
Chair: J. Oscar Croxatto, MD
Miguel Materin, MD
Arturo Irarrazaval, MD
David Pelayes, MD
Guillermo Chantada, MD
Marcelo Zas, MD
Adriana Fandiño, MD
EmilianoBecerra, MD
Ignacio Zeolite, MD
Carolina Gentile, MD

List of Congress Meetings of the International Society of Ocular
Oncology
I
II
III
IV
V
VI
VII
VIII
IX
X
XI
XII
XIII
XIV
XV
1977 Cambridge (England)
1981 Schwerin (former East Germany)
1984 Nylon (Switzerland)
1987 Zusdel/Moskow (Russia)
1989 Essen (Germany)
1992 New York (USA)
1994 Florence (Italy)
1997 Jerusalem (Israel)
1999 Philadelphia (USA)
2001 Amsterdam (The Netherlands)
2003 Hyderabad (India)
2005 Whistler (Canada)
2007 Siena (Italy)
2009 Cambridge (England)
2011 Buenos Aires (Argentina)
3

Patronage
Agencia Nacional de Promoción Científica y Tecnológica
4

Table of Contents
Welcome address
Keynote Lectures
The Stallard Lecture
General Information
Buenos Aires MAP
Program Overview
Research Day
Presentations
Abstracts
Retinoblastoma
Presentations
Abstracts
Posters
Poster Abstracts
Eyelid, Conjuntiva, Orbit and Other Intraocular Tumors
Presentations
Rapid Fire Cases
Posters
Abstracts
Rapid Fire Abstracts
Poster Abstracts
Uveal Melanoma
Presentations
Posters
Abstracts
Posters Abstracts
Index (First Authors)
5
7
8
9
10
12
13
15
18
29
35
32
48
61
62
65
67
75
83
91
94
96
108
118

Dear Friends,
Welcome Address
ISOO Committee
Welcome to the 15th meeting of the International Congress of Ocular Oncology, the fifth one as the official congress of the
International Society of Ocular Oncology. Our Society has made progress in working as an organization in recent years, and for the
first time the preparations for our biennial meeting including membership and registration payments and submission of abstracts
were done through the new website of our Society.
Our Society continues to grow, and we have members from many countries throughout the world. This is the first time
that the International Ocular Oncology meeting is being held in Latin America. This fact reflects our goal to expose our specialty to
all parts of the world.
I welcome all those from Latin American countries who have an interest in ocular oncology to join us in our meeting and to
join our Society.
The basic research day, which started as an annex at our meeting in Amsterdam in 2001, has become an integral part of
our general meeting. I’m happy that basic researchers are interested in participating in our meetings, which have become more and
more scientific.
I wish to thank the local organizing committee, headed by J. Oscar Croxatto, who worked so hard to take care of all the
logistics of our meeting, and for planning the social program. I want to thank Martine Jager, head of the program committee, for all
her efforts in planning the scientific program and to Bill Harbour for organizing the research day. I would also like to thank Bertil
Damato, vice president, and Tero Kivela, secretary of the ISOO for their advice, and a special thanks to Shahar Frenkel for his hard
work in establishing and running our new website.
I wish all of you interesting scientific sessions and enjoyable social functions. Please take this opportunity of the congress
being held in the beautiful City of Buenos Aires to meet friends, learn from each other, and plan collaborations for future clinical and
basic studies in our so very special field of ocular oncology.
Jacob Pe’er, M.D.
President
International Society of Ocular Oncology
Dear Members and Guests,
Local Committee
In representation of the Local Organizing Committee, I would first of all like to offer you our warmest personal greetings
and welcome you to Buenos Aires, Argentina. We are both proud and excited to host the XV Biannual Meeting of the International
Society of Ocular Oncology.
We express our gratitude to all scientists, ophthalmologists and oncologists from all over the world traveling to South
America, and the support of all members of ISOO for having selected Buenos Aires to host this biennial meeting. Especially, we
would like to thank Dr. Jacob Pe’er, President of ISOO for his enthusiasm and continuous guidance, Dr. William Harbour for the
organization of an exciting research day, Dr. Jerry Shields and his fervor on “rapid-fire cases”, Dr. Martine Jager for putting together
the whole program, and none the less, Dr. Shahar Frenkel, who did a strenuous work in the arrangement and maintenance of the
web page, and gathering all registrations and submitted abstracts.
Scientific sessions contain a compressed range of topics on ocular oncology, including four keynote lectures, The Stallard Medal
Lecture, 122 paper presentations, 85 posters, and 35 brief case reports. The expectations of the meeting are high. New diagnostic
tools, advances therapies and results of creative and in deep molecular biology research in tumors of the eye and ocular adnexa will
evolve in better therapies and prognosis.
We would like to take this opportunity to thank all those who have contributed to make this event possible. We express
our sincere wish that the exchange of ideas and opinions at the conference will benefit scientists, physicians and patients.
With our warmest regards,
Juan Oscar Croxatto, MD
Chair
Local Organizing Committee.
7

Keynote Lectures
Lymphoma of the ocular adnexa
Steffen Heegaard, MD
Professor of Ophthalmology, University of Copenhagen, Denmark.
Steffen has worked mostly with characterising ocular adnexal lymphoma clinically, pathologically and genetically.
The latest results regarding lacrimal gland lymphoma will be presented in the key note lecture. Also the results of
the latest treatment modalities will be given.
Intraocular melanoma and their metastatic pattern have been described and the invasion pattern into the optic
nerve head has been characterised along with the description of so called neurophilic intraocular melanomas. Also
Melanoma Associated Spongioform Scleropathy has been characterised by the different metalloproteinases and
the possible correlation to extraocular extension of malignant melanoma.
Clinical implications of GNAQ and GNA11 mutations in Uveal Melanoma
Emine Kilic, MD, PhD
Ophthalmology Erasmus MC Rotterdam.
Dr Emine Kilic (Ocular Oncologist and Vitreoretinal surgeon) holds a position as a staff member in the Ophthalmology
department of the Erasmus MC University Hospital, Rotterdam in the Netherlands. She completed her PhD on
genetics of uveal melanoma at the Clinical Genetics and Ophthalmology departments of the Erasmus University
and did her residency in Ophthalmology in the Erasmus MC. She has a special interest in the genetics of Uveal
Melanoma and is a senior researcher in the Rotterdam Ocular Melanoma Studygroup (ROMS). ROMS research
has focussed on the cytogenetic and molecular genetic evaluation of genetic changes in Uveal Melanoma, in
particular on aberrations of chromosomes 1, 3, 6 and 8 and expression profiling of a well-selected subpopulation
of genetically well-characterized tumours. With the availability of newer and more specific techniques for studying
Uveal Melanoma these are further explored and applied for this group of patients. Besides this she is involved in the
study on etiologic factors that may be important in the pathogenesis of Uveal Melanoma and their role in tumour
progression and metastasis.
Insights From Sequencing the Melanoma Exome
Ruth Halaban, PhD
Department of Dermatology, Yale University School of Medicine,
New Haven, CT, USA
Ruth Halaban, a Senior Research Scientist/Professor at Yale University School of Medicine, is an established
scientist in the field of pigmentation and melanoma research. Her main scientific contributions are growth factors/
receptors regulating the proliferation and differentiation of normal melanocytes, aberrant autonomous growth
of melanoma cells, molecular biology of melanocyte specific genes, mechanisms of malignant transformation,
epigenetic regulation, signal transduction and drug responses. More recently, she and her colleagues sequenced
~100 melanoma exomes. The results of this effort are now being investigated in the context of melanocyte
transformation, oncogenes/tumor suppressor genes and melanoma cell heterogeneity. Her prominence in the field
of melanocyte and melanoma biology is reflected by her authoring over 121 publications in peer-reviewed journals,
including 19 solicited reviews, and her receipt of numerous awards, such as the 2009 Lifetime Achievement Award
in melanoma research granted by the Society of Melanoma Research and this year Myron Gordon medal by the
Inrerbational Federation of Pigment Cell Societies.
8

Intravitreal injection in retinoblastoma revisited: from prohibition
to conditional indications
Francis Munier, MD
Head of Retinoblastoma and Oculogenetic Units, Jules Gonin Eye
Hospital, Lausanne, Switzerland.
Prof. Francis Munier studied at the University of Lausanne Medical School in Switzerland. On top of clinical
training at Jules Gonin Eye Hospital and Division of medical Genetics in Lausanne, he received additional training
in Pediatric Ophthalmology and Ocular Oncology at Childrens Hospital Los Angeles, University of Southern
California in USA. Professor Munier is board certified in both Ophthalmology and Medical Genetics. He is presently
head of the the Retinoblastoma Unit and the Oculogenetic Unit at Jules Gonin Eye Hospital. In addition Prof.
Munier is an associated researcher at the Institut de Recherche en Ophtalmologie (IRO) in Sion, Switzerland.
The Stallard Medal
Anterior Segment Surgery of Intraocular Tumors
Enrique S. Malbran, MD
Director, Clínica Oftalmológica Malbran, Buenos Aires, Argentina.
Dr. Enrique S. Malbran is one of the most prestigious surgical and clinical ophthalmologists with a wide area
of expertise. He founded and is currently President of the Fundación Oftalmológica Jorge Malbran. He holds a
chair at the National Academy of Medicine of Buenos Aires, and is a member of the Academia Ophthalmologica
Internationalis, Concilium Ophtalmologicum Universale, among many other national and international societies.
He delivered numerous named lectures including the 28th Edward Jackson Memorial Lecture in 1971, and received
many distinctions and awards around the world. The themes of his original contributions, to mention a few, comprise
corneal surgery, cataract surgery, vitreoretinal surgery, and surgery of intraocular tumors.
9

The venue for the Biannual Meeting of the International Society of
Ocular Oncology (ISOO) is the nH City Hotel & Tower, Bolivar 160/120
Ciudad Autónoma de Buenos Aires.
The intended audience for the Biannual Meeting ISOO 2011 of ocular
oncology program are ophthalmologists, medical oncologists, pediatric
oncologists, radiation oncologists, radiation physicists, radiologists,
pathologists, pharmacologists, molecular biologists, and geneticists
who are engaged in the diagnosis and treatment of tumors of eye and
ocular adnexa.
The objective of the International Society of Ocular Oncology and the
Biennial Meeting ISOO 2011 is to provide opportunities for the free
expression and interchange of ideas and information for educational
purposes. Neither the ISOO nor the Local Committee of the meeting
accepts responsibility for any opinions, positions, or statements
contained or expressed in such material and such opinion, positions or
statements are not necessary those of the ISOO or the Local Committee
of the meeting.
LANGUAGE
English is the official language of the meeting. Translation will not be
provided.
ATTENDEE REGISTRATION
Attendees which did not register in advance may register on-site. The
On-site Registration Area will be staffed from Monday to Thursday,
November 14, from 7:30 am to 4:00 pm.
Forms of Payment
Forms of payment that will be accepted will be cash and credit cards
in Argentine pesos and US dollars. Forms of payment that cannot be
accepted are personal checks, traveler’s checks, or other cash currencies
that are not Argentine pesos or US dollars.
On-site Registration Fees (US Dollars)
Active Member US$ 935
Associate/ Non- member US$ 1035
Welcome Cocktail (only companions) U$S 50
Tango Show & Dinner (per person) U$S 175
Gala Dinner (per person) U$S 150
The registration fee is for all scientific events. There are no additional
course fees. .
Badges and Replacement of Lost Badges
Your badge is your pass to enter the exhibit areas, scientific sessions
and social events. Please wear it at all times. Replacement of badges is
available at the On-site Registration Area. There is a $50 replacement
fee for lost badges. Badges are not transferable and cannot be lent to
anyone for any purpose during the Biennial Meeting ISOO 2011. Altered
badges (including attaching business cards) will be confiscated by
security guards.
Children
Children ages 17 and under receive a complimentary registration and
may enter the Exhibit Area only if accompanied by a registered adult.
Registration for children in this category must be done at the On- Site
Registration Area in the foyer of the Gaudi Room of the nH City & Tower
Hotel.
Proof of Attendance Certificate
Attendees of the Biennial Meeting ISOO 2011 have to ask for a certificate
of attendance in the registration area.
General Information
10
SCIENTIFIC SESSIONS
SPEAKER READY ROOM
Gaudi Room
To assist program participants with their audiovisual needs, the Speaker
Ready Box Room within the Gaudi Room, is staffed from Monday to
Thursday, November 14-17. Scientific session presenters must submit
their Power Point presentations to this room at least three working
hours before their presentations. Early morning presenters should
submit their material before 4:00 pm on the preceding day. PowerPoint
presentations may be previewed in this room, and videotapes may be
cued to the section to be shown.
Buenos Aires: A brief historical profile
The Argentine Republic is the most extensive country in the Spanishspeaking
world, in the extreme south of Latin America, and constitutes a
vast triangle situated in the Andes mountain range. The actual Argentine
territory was colonized by Spain at the beginning of the 16th century.
In 1810, as a result of the Napoleonic occupation of the Kingdom of
Spain, Buenos Aires started the patriotic independence on the 9th of
July 1816.
The name Argentina comes from the Latin “Argentum” which means
silver in the sense of “Country of Silver.” The origin of this term dates
back to the arrival of the first conquistadors to the Rio de la Plata. The
men who were shipwrecked in the expedition of Juan Diaz de Solís,
the discoverers of this river “the sweet sea,” were to meet with the
indigenous inhabitants who presented them with silver objects. These
items were taken to Spain until 1524, along with tales of a fabulous
mountain rich in the metal, the land of silver. From that moment, they
called the Solís River “Rio de la Plata” or the Silver River. In 1820,
President decreed that the government adopted the name “Argentine
Republic.”
Buenos Aires and its surroundings
The city of Buenos Aires is situated on the banks of Rio de la Plata.
This immense estuary, the widest in the world, gives this metropolis a
vision of the infinite. Buenos Aires offers the visitor an attractive cultural
pluralism, from the eclectic nature of its architecture to the varied ethnic
nature of its inhabitants or the wealth of its gastronomy.
The cultural heritage includes the Colon Theatre, a historical national
monument, and one of the great concert halls in the entire world. It
also includes theatres such as the Coliseum, Cervantes, General San
Martín and President Alvear; also impressive are the cultural centers in
Recoleta, Ricardo Rojas and Jorge Luis Borges. Moreover, there are 80
museums, more than a hundred art galleries, hundreds of bookshops,
24 libraries and a Planetarium.
There are also a number of antiques shops, elegant shopping centers
and a great number of boutiques catering to sophisticated tastes.
Nightlife is particularly varied, especially in the city center where there
are the most important nightclubs, discotheques, cafes and places to
dance tango or to enjoy folkloric music.
Parks, squares, avenues and districts of many different kinds, each with
a distinct idiosyncrasy, extend from the center to the south, the north
and the west: the areas of San Telmo, la Boca, Palermo or Belgrano,
Lezama Park, Puerto Madero, Plaza San Martin, Plaza Lavalle, Plaza del
Congreso, Recoleta, the avenues of Mayo, 9 de Julio, Santa Fe, Alvear
or pedestrian street Florida and many other places which give to the
cosmopolitan capital of Argentina, in its different manifestations, a soul
that is different and unmistakable. As Borges said, “a silent magic which
bewitches almost completely all those who come to visit her.”

Climate
In November, the weather of Buenos Aires is generally pleasant,
with sunny days and nights still fresh. The average temperature is
20.3°C/68.5 F.
Clothing
Since it is spring in Buenos Aires, bring appropriate clothing, especially
for going out at night. All varieties of attire are used in Buenos Aires,
from casual attire to gala attire.
Currency
The Argentine currency is the peso. However, American dollars are also
accepted in the majority of shops and restaurants for conveniences.
American dollars and traveler’s cheques can be exchanged at banks
and hotels at the daily exchange rate.
Tipping
10% to 15% of the bill is considered a reasonable tip for good
service. In Argentina, the tip is almost never included in the check.
Always pay the tip in cash separately from the check; for example,
when paying the check with credit card, do not include the tip in
the total amount.
Electricity
The electric current is 220 volts AC. For travelers coming from countries
where 110 volts AC/60 cycles is common, be sure to protect your
equipment from damage.
Bank and Money Exchange
Cash machines are located in several locations. You can always
exchange money at the front desk of your Hotel.
11
Drinking Water
It is always wiser to drink bottled water, although most hotels and
establishments have purified water.
Public Transportation
Buenos Aires boasts of an extensive public transportation system of
buses and a metro system. Please inquire at the front desk of your Hotel
for detailed tourist information.
Taxis & Airport Transportation
Taxis from the Ezeiza International Airport to downtown coasts
approximately between 40 US dollars (130 pesos). When traveling about
the city ask for a taxi in the lobby of your hotel. Do not accept rides from
unmarked taxis.
SOCIAL EVENTS
Opening Ceremony & Cocktail
Tango Show & Dinner
Gala Dinner,
SAFETY TIPS
ALWAYS remove your badge when leaving your Hotel.
RIDE rather than walk, especially at night.
AVOID unlighted driveways and other shadowed places.
WALK with another person when sightseeing or shopping, especially at night.
ASK at the front desk or the concierge desk about the neighborhood
around your hotel.
BE WARY of strangers. Never accompany a stranger anywhere.
REMAIN ALERT at all time.
DON´T TAKE unmarked taxis or accept a ride from anyone you don’t know.
DON´T WALK through dark, unattended parking lots or other desert area.

Buenos Aires Downtown MAP
12
Venue

Monday, November 14
Tuesday, November 15 Wednesday, November 16 Thursday, November 17
RESEARCH DAY
RETINOBLASTOMA EYELID, CONJUNCTIVA & ORBIT UVEAL MELANOMA
8:30 hs Papers Poster Presentations (Rbp 100- Poster Presentations (ECOp Poster Presentations (Ump
120)
101-111)
101-113)
9:00 hs Papers
9:20 hs Papers
9:30 hs Papers
10:10 hs Break
10:20 hs Break Break
10:40 hs Keynote Lecture
10:50 hs Emine Kilic, MD PhD Papers Stallard Lecture
11:00 hs Break Prof. Enrique S. Malbran
11:05 hs Papers
11:20 hs Papers Papers
12:00 hs Keynote Lecture Poster Presentations (Ump
114-126)
Program Overview
12:25 hs Ruth Halaban, MD PhD Keynote Lecture
12:30 hs Lunch Francis L. Munier, MD Lunch
12:45 hs Poster Presentations (OTp 101-
110)
12:50 hs Lunch
13:15 hs Lunch
14:00 hs Panel Discussion Papers
13
14:10 hs Poster Presentations (Rbp 121-
138)
14:15 hs Keynote Lecture
Steffen Heegard, MD
14:35 hs Rapid Fire Cases
14:45 hs Papers
14:50 hs Papers
15:40 hs Break
15:45 hs Break
16:00 hs Break
16:10 hs Papers
16:15 hs Papers
16:20 hs Papers
17:00 hs Discussion
17:20 hs Business Meeting
17:30 hs
17:35 hs
18:20 hs

MORNING
8.30-10.10 Papers
(Moderators: D. Wilson, H. Grossniklaus)
1836 RES 1
GENE EXPRESSION DIFFERENCES BETWEEN
MONOSOMY 3 AND DISOMY 3 UVEAL MELANOMA
Arun D. Singh, Nakul Singh, Gurkan Bebek, Charis Eng, Raymond Tubbs,
Yogen Saunthararajah, Pierre Triozzi
2242 RES 2
CORRELATION BETWEEN GEP AND OTHER
PROGNOSTIC FACTORS FOR METASTATIC DEATH IN
205 PATIENTS WITH POSTERIOR UVEAL MELANOMA
EVALUATED BY FNAB
Zélia M. Corrêa, James J. Augsburger, J. William Harbour
2227 RES 3
MOLECULAR GENETICS MUTATION PROFILES OF
UVEAL MELANOMA METASTASES ASSOCIATED WITH
SURVIVAL
Colleen M. Cebulla, Benjamin N. Christopher, Vishal Verma, Dominic
Buzzacco, Thomas Olencki, Frederick H. Davidorf, Mohamed H. Abdel-
Rahman
2226 RES 4
NOTCH SIGNALING PROMOTES UVEAL MELANOMA
GROWTH
Charles Eberhart, Katayoon Ebrahimi, Karisa Schreck, Eli Bar, J. William
Harbour James Handa, Shannath Merbs, Laura Asnaghi
1624 RES 5
IN VIVO CONTRAST-ENHANCED HIGH-FREQUENCY
ULTRASONOGRAPHY OF UVEAL MELANOMA
IN ANIMAL MODELS: IMAGING FEATURES AND
HISTOPATHOLOGIC CORRELATIONS
Hans E. Grossniklaus, Qing Zhang, Hua Yang, Shin J. Kang, Yanggan
Wang, Tonya Coulthard
21 RES 6
HISTOPATHOLOGICAL AND DOSIMETRIC FINDINGS
IN A PORCINE MODEL OF OCULAR RADIATION INJURY
FROM IODINE-125 PLAQUE BRACHYTHERAPY
Scott C. N. Oliver, Victor Hsu, Lucy Bollinger, Moyed Miften, Laurie Gaspar,
Philip Boyer
2347 RES 7
USE OF A MICROCATHETER TO OBTAIN BIOPSIES
FROM UVEAL MELANOMA
David J. Wilson, William J. Harbour
RESEARCH DAY
Monday November 14, 2011
15
148 RES 8
EVALUATION OF HISTONE H3K4 METHYLATION BY
IMMUNOHISTO-CHEMISTRY AND RELATIONSHIP TO
AGGRESSIVE UVEAL MELANOMA
L. Schoenfield, M. Turell, P. Carver, R. Tubbs, A. Singh, Y.
Saunthararajah
42 RES 9
ROLE OF THE CHEMOKINE RECEPTOR CXCR4 IN
THE DEVELOPMENT AND PROGRESSION OF UVEAL
MELANOMA
Shahar Frenkel, Ariela Miller, Amnon Peled, Jacob Pe’er
1703 RES 10
EXPRESSION OF MACROPHAGE-ATTRACTION
MOLECULES IN UVEAL MELANOMA: INDUCTION BY
HYPOXIA?
I.H.G. Bronkhorst, M. Versluis, G.P.M. Luyten, P.A. van der Velden, M.J.
Jager
10.10-10.40 BREAK
10.40-11.05
Keynote Lecture: Dr. Emine Kilic
CLINICAL IMPLICATIONS OF GNAQ AND GNA11
MUTATIONS IN UVEAL MELANOMA
11.05-12.00 Papers
(Moderators M. Materin, Z. Correa)
525 RES 11
ONCOGENE MUTATION PROFILING IN OCULAR
MELANOMA
Ivana K. Kim, Laura MacConaill, Emanuele Palescandolo, Shan Lu, Joo-
Eun Lee, Scott Adams, Margaret M. DeAngelis, Paul Van Hummelen,
Anthony Daniels, Levi Garraway, J. William Harbour, Evangelos S.
Gragoudas
1958 RES 12
DETECTION OF GNAQ/GNA11 MUTATIONS IN
CIRCULATING CELL-FREE DNA AS A BIOMARKER OF
UVEAL MELANOMA
Jordan Madic, Bénédicte Trouiller, David Gentien, Maud Milder,
Stéphanie Saada, Franck Assayag, Fariba Nemati, Didier Decaudin,
Laurence Desjardins, Sophie Piperno-Neumann, Olivier Lantz, Marc-
Henri Stern

2109 RES 13
GNAQ/11 MUTANT DEPENDENT UVEAL MELANOMA -
SENSITIVITY TO MEK AND PI3K INHIBITION
Scott E. Woodman, Xiaoxing Yu, Jahan Khalili, Chandrani Chattopadhyay,
Michelle Williams, Nancy Poindexter, Martine J. Jager, Elizabeth Grimm,
Dan Gombos, Bita Esmaeli
149 RES 14
INACTIVATING MUTATIONS IN BAP1 IN METASTASIZ-
ING CLASS 2 UVEAL MELANOMAS
J. William Harbour, Michael D. Onken, Lori A. Worley, Anne M. Bowcock,
Eli Roberson
1944 RES 15
A RECURRENT DELETION OF THE BAP1 LOCUS IN
OCULAR MELANOMA
Suresh C. Jhanwar, Yuqiang Fang, Lu Wang, Klaus Busam, David H.
Abramson
1843 RES 16
BAP 1 MUTATION IN TWO FAMILIES WITH UVEAL
MELANOMA
Miguel A. Materin, Camila Simoes, Ruth Halaban, J. William Harbour
12.00-12.30
Keynote Lecture: Dr. Ruth Halaban
INSIGHTS FROM SEQUENCING THE MELANOMA EXOME
12.30-14.00 LUNCH
AFTERNOON
14.00-14.45 Panel discussion Uveal Melanoma
(Moderator J.W. Harbour)
14.45-15.45 Papers
(Moderator D. Abramson)
103 RES 17
IDENTIFICATION OF MOLECULAR SUBGROUPS OF
RETINOBLASTOMA
M. Parulekar, G. Kapatai, M.A. Brundler, A. Peet, M. Wilson, H. Jenkinson,
C. McConville
1820 RES 18
A NEW DISEASE: RETINOBLASTOMA WITH NO
DETECTABLE RB1 MUTATIONS DRIVEN BY MYCN
AMPLIFICATION
Brenda L. Gallie, D.E. Rushlow, S. Yee, J.Y. Kennett, P. Boutros, N.L.
Prigoda-Lee, W. Halliday, S. Pajovic, C. Spencer, B.L. Thériault, H.
Dimaras, A. Raizis, C. Houdayer, W.L. Lam, T. Corson, D. Lohmann
RESEARCH DAY
Program
16
39 RES 19
TARGETING THE P-53 PATHWAY IN RETINOBLAS-
TOMA PRECLINICAL MODELS WITH SUBCONJUNC-
TIVAL NUTLIN-3A
Rachel C. Brennan, Sara Federico, Cori Bradley, Jiakun Zhang, Jacqueline
Flores-Otero, Matthew Wilson, Clinton Stewart, Fangyi Zhu, Kip Guy,
Michael A. Dyer
1656 RES 20
RB DEPLETION SELECTIVELY INDUCES PROLIFERATION
OF CONE-PRECURSOR LIKE CELLS
David Cobrinik, Xiaoliang L. Xu, Suresh C. Jhanwar, and David H.
Abramson
615 RES 21
TRB2 AND SKP2 IN THE RETINOBLASTOMA PATHWAY
Xiaoliang L. Xu, Renbing Jia, Nengyi Zhou, Xianqun Fan, David H.
Abramson, David Cobrinik and Suresh C. Jhanwar
1515 RES 22
NESTED RT-PCR DETERMINATION OF GD2
SYNTHASE AS A MARKER FOR MINIMAL DISEASE IN
RETINOBLASTOMA IN THE CEREBRO-SPINAL FLUID
(CSF).
Viviana Laurent, Claudia Sampor, Jorge Rossi, Mariano Gabri, Maria T.G.
de Davila, Daniel Alonso, Guillermo Chantada
15.45-16.15 BREAK
16.15-17.00 Papers (moderators R. Hurwitz, G.
Chantada)
1824 RES 23
SUPER-SELECTIVE INTRA-OPHTHALMIC ARTERY
CHEMOTHERAPY: RETINAL ENDOTHELIAL TOXICITY
IN PRE-CLINICAL MODELS
Matthew W. Wilson, J. Scott Williams, John S. Jackson, Fan Wang, Clinton
F. Stewart, Timothy D. Mandrell, Barrett G. Haik, Jena J. Steinle
2107 RES 24
EMBRYONIC RETINAL TUMORS IN TRANSGENIC MICE
CONTAIN CD133+ TUMOR-INITIATING CELLS
Richard L. Hurwitz , Lalita Wadhwa, Wesley Bond, Laszlo Perlaky, Paul
Overbeek, Mary Y. Hurwitz, Patricia Chévez-Barrios
2333 RES 25
TREATMENT OF LHBETATAG RETINOBLASTOMA
TUMORS WITH ANGIOGENIC AND GLYCOLYTIC
INHIBITORS AVOIDS CHEMOTHERAPY
Samuel K. Houston, Timothy G. Murray, Yolanda Pina, Christina Decatur

50 RES 26
USING THE GLYCOLYTIC INHIBITOR 2-FLUORODEOXY-
D-GLUCOSE, A NOVEL APPROACH TO TARGET THE
CHEMORESISTANT CELL POPULATION IN LHBETATAG
RETINAL TUMORS
Yolanda Piña, Christina L. Decatur, Samuel Houston, Timothy G. Murray,
Ludimila Cavalcante, and Theodore Lampidis
2140 Res 27
MTOR TARGETING IN COMBINATION WITH
CHEMOTHERAPY: UPSTREAM REGULATION
OF ANAEROBIC GLYCOLYSIS TO TARGET THE
CHEMORESISTANT CELL POPULATION IN RB
Timothy G. Murray, Yolanda Piña, Christina L. Decatur, Samuel Houston,
Ludimila Cavalcante, and Theodore Lampidis
17.00-17.30 Discussion
(moderator B. Gallie)
RESEARCH DAY
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17

1836 RES 1
GENE EXPRESSION DIFFERENCES BETWEEN MONO-
SOMY 3 AND DISOMY 3 UVEAL MELANOMA
Arun D Singh, Nakul Singh, Gurkan Bebek, Charis Eng, Raymond
Tubbs, Yogen Saunthararajah, Pierre Triozzi. Cole Eye Institute, Human
Genomics Institute, Taussig Cancer Center, Cleveland Clinic Foundation
(singha@ccf.org)
Purpose. Presence of monosomy 3 in uveal melanoma has been
associated with metastatic disease. We aim to characterize the gene
expression differences between disomy 3 and monosomy 3 uveal
melanoma tumor samples.
Methods. The gene expression profile of 24,357 genes from 57 uveal
melanomas tumor samples were measured (Illumina HumanRef8-
Version 3). Copy number status was assessed by SNP microarray
(Illumina 660W V1) and FISH. 38 samples were found to have monosomy
3 or uniparental disomy and 19 samples were disomic. Raw gene
expression data was transformed by variance stabilizing and robust
spline normalized. Finally, gene expression differences were calculated
using the LIMMA package in R.
Results. In total, 622 genes were found to be differentially expressed;
with 43 overexpressed genes (2 fold) and 49 under expressed genes
(2 fold) in monosomy 3 tumors. When enriched for gene ontology
(GO) annotations, GO 0048856 [anatomical structural development]
and GO 0042273 [ribosomal large subunit biogenesis] were found
to be statistically significantly overrepresented. PTP4A3 and ENPP2
[autotaxin] have been previously implicated in uveal melanoma, lending
credibility to list.
Conclusions. Analysis of most differentially expressed genes indicates
that several pathways are involved in pathogenesis of metastatic uveal
melanoma, suggesting heterogeneous nature of the tumor. Furthermore
the list of differentially expressed genes provides potential therapeutic targets.
Financial disclosure. None
2242 RES 2
CORRELATION BETWEEN GEP AND OTHER
PROGNOSTIC FACTORS FOR METASTATIC
DEATH IN 205 PATIENTS WITH POSTERIOR
UVEAL MELANOMA EVALUATED BY FNAB
Zélia M. Corrêa, MD, PhD, James J. Augsburger, MD, J. William Harbour
MD (correazm@uc.edu)
Department of Ophthalmology, University of Cincinnati College of
Medicine, Cincinnati, Ohio, USA
Department of Ophthalmology, Washington University School of
Medicine, St. Louis, Missouri, USA
Purpose. Determine the relationships between gene expression profile
(GEP) class, cytologic diagnosis, and size of melanocytic uveal tumors
sampled by FNAB, and comment on the overall survival of patients.
Methods. 205 patients with a primary posterior uveal melanoma
underwent prognostic FNAB as part of a collaborative multicenter
prospective study. In each case, at least 2 aspirates were obtained and
submitted for both cytopathologic and GEP. Relationships between GEP,
cytopathologic classification, tumor size, and intraocular tumor location
were studied by cross tabulation analysis. Survival was computed
using the Kaplan-Meier method. Significance of differences between
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Abstracts
18
subugroups was evaluated using the logrank test.
Results. 75% of patients 55 years of age or younger had a GEP class 1
tumor. This proportion decreased with older age. Patients with smaller
tumors (LBD ≤ 10mm) had a higher percentage of class 1 tumors (79.2%)
compared to those with larger tumors (LBD > 15 mm) (48.6%). 75%
of purely choroidal tumors were GEP class 1 while more than 50% of
ciliary body tumors were GEP class 2. The relationship between GEP
class 1 and low-grade cytology was present but did not reach statistical
significance. FNAB specimen was insufficient for cytodiagnosis in 43
cases (20.1%), and for GEP in only one case (0.5%). Currently, 189 of
205 patients are alive without metastasis (92.2%). Cumulative actuarial
survival based on deaths from metastatic uveal melanoma in the entire
group was 79.7% at three years. Three-year cumulative actuarial survival
was 95.7% in the GEP Class 1 subgroup versus 59.6% in the GEP Class 2
subgroup (p = 0.007, logrank test).
Conclusions. GEP was both (1) substantially more robust discriminator
between surviving patients and those dying of uveal melanoma
metastasis than any other evaluated variable and (2) much more likely
to yield a definitive prognostic classification than cytopathology.
Financial disclosure. NEI grant EY02687, NCI grant R01CA125970, Kling Family Foundation,Tumori
Foundation, Barnes-Jewish Hosp Foundation, Horncrest Foundation, Research to Prevent
Blindness, Quest for Vision Fund-U Cincinnati
2227 RES 3
MOLECULAR GENETICS MUTATION PROFILES OF
UVEAL MELANOMA METASTASES ASSOCIATED WITH
SURVIVAL
Colleen M. Cebulla1, Benjamin N. Christopher1, Vishal Verma1, Dominic
Buzzacco1, Thomas Olencki3, Frederick H. Davidorf1, Mohamed H.
Abdel-Rahman1,2 (colleen.cebulla@osumc.edu)
1. Department of Ophthalmology; 2. Division of Human Genetics,
Department of Internal Medicine; 3. Medical Oncology Program, The
Ohio State University, Columbus, OH
Purpose. To investigate the molecular genetics status of uveal melanoma
(UM) metastases and correlate it with disease progression.
Methods. IRB approval was obtained. Twelve pathologically confirmed
UM metastases from 11 patients were included. Molecular genetic
alterations in chromosomes 3, 8q, 6p, and 1p, and the BAP1 gene region
were investigated by microsatellite genotyping. Mutations in codons 183
and 209 of GNAQ and GNA11 genes were studied by restrictive fragment
length polymorphism (RFLP).
Results. Patients with monosomy 3 tumours (4/11) died rapidly with
metastatic disease (mean survival = 5 months, range 1-8 months).
In contrast, patients with disomy or partial chromosome 3 tumour
alterations showed significantly slower metastatic disease progression
(mean survival = 69 months, range 40- 123 months, p=0.003).
Alterations in chromosomal arms 1p (5/10), 6p (6/10), and 8q (9/11),
BAP1 LOH (8/11), and mutations in either GNAQ or GNA11 (8/11) were
not differentially associated with survival. Prominent mononuclear
inflammatory infiltrate was observed in tumours from patients with
slowly progressive disease.
Conclusions. In UM metastases, monosomy 3 is associated with highly
aggressive, rapidly progressive disease while disomy or partial change
of 3 and prominent mononuclear inflammatory infiltrate in the tumour is
associated with better prognosis. These findings should be considered
when designing clinical trials testing effectiveness of various therapies
of metastatic UM.
Financial disclosure. None

2226 RES 4
NOTCH SIGNALING PROMOTES UVEAL MELANOMA
GROWTH
Eberhart Charles, Ebrahimi Katayoon, Schreck Karisa, Bar Eli, Harbour J,
William Handa, James, Merbs Shannath, Asnaghi Laura (ceberha@jhmi.edu)
Departments of Pathology, Ophthalmology and Oncology, Johns Hopkins
University, School of Medicine, Baltimore, MD, USA
Department of Ophthalmology & Visual Sciences, Washington University
School of Medicine, St. Louis, MO, USA
Purpose. To determine if uveal melanoma requires Notch activity for
growth and metastasis.
Methods. Expression of Notch pathway members was characterized
in primary tumor samples and in cell lines using Western blots, real
time RT PCR and gene expression arrays. Notch inhibition was achieved
using small molecule inhibitors and shRNA constructs targeting multiple
pathway members. Notch signaling was induced with constitutively active
truncated receptors. Growth, invasion and clonogenicity were measured
in vitro using standard assays, and in vivo xenografts were examined
histopathologically.
Results. Notch receptors, ligands and targets were expressed in all five cell
lines examined, and in 30 primary uveal melanoma samples. Interestingly,
the three lines with high levels of baseline pathway activity (OCM1, OCM3,
OCM8) had their growth reduced by pharmacologic Notch blockade using
the gamma-secretase inhibitor (GSI) MRK003. In contrast, two uveal
melanoma lines (Mel285, Mel290) with very low expression of Notch
targets were insensitive to the GSI. Constitutively active forms of Notch1
and Notch2 promoted growth of uveal melanoma cultures and were able
to rescue the inhibitory effects of GSI. MRK003 treatment also inhibited
anchorage-independent clonogenic growth and cell invasion, and reduced
phosphorylation levels of Akt and Erk1/2. Suppression of canonical Notch
activity using shRNA targeting Notch2 or CBF1 was also able to reduce
tumor growth and invasion. Notch signaling was required to maintain
Twist1 expression in uveal melanoma cells, and Twist1 knockdown using
shRNA reduced invasion. Finally, intraocular xenograft growth was
significantly reduced by GSI treatment.
Conclusions. Our findings suggest that Notch plays an important role in
inducing proliferation and invasion in uveal melanoma, and that inhibiting
this pathway may be effective in preventing tumor growth and metastasis.
Financial disclosure. None
1624 RES 5
IN VIVO CONTRAST-ENHANCED HIGH-FREQUEN-
CY ULTRASONOGRAPHY OF UVEAL MELANOMA
IN ANIMAL MODELS: IMAGING FEATURES AND
HISTOPATHOLOGIC CORRELATIONS
Hans E. Grossniklaus1, Qing Zhang1, Hua Yang1, Shin J Kang1, Yanggan
Wang1, Tonya Coulthard2 (ophtheg@emory.edu)
1. Emory University School of Medicine, Atlanta, Georgia
2. Visual Sonics, Toronto, Canada
Purpose. To evaluate the utility of in vivo imaging of a mouse model
of uveal melanoma utilizing high-frequency contrast-enhanced
ultrasound (HF-CE-US) with 2- or 3-dimensional modes, and to correlate
the sonographic findings with histopathologic characteristics.
Methods. Fourteen 12-week-old C57BL6 mice were inoculated into
RESEARCH DAY
Abstracts
19
their right eyes with aliquots of 5X105/2.5µL of B16LS9 melanoma
cells, and randomly assigned into 2 groups. At 7 days post inoculation,
tumor-bearing eyes in group 1 (n = 8) were imaged using high
frequency ultrasound with microbubble contrast agent to determine
the 2-dimensional tumor size and relative blood volume; eyes in
group 2 (n = 6) were imaged by 3-dimensional mode ultrasound with
microbubble enhancement, and the tumor volume was determined.
Histologic tumor burden was quantified in enucleated tumor-bearing
eyes by Image J software, and microvascular density was determined
by counting vWF-positive vascular channels. The 2D or 3D in vivo
imaging were evaluated and compared with histopathologic findings.
A rabbit model of ocular melanoma was also evaluated.
Results. Utilizing high frequency ultrasound with microbubble contrast
agent in the mouse model, melanomas were visualized as relatively
hyperechoic regions in the images. The intraobserver variability was
9.65 ± 7.89% and the coefficient of variation for multiple measurements
was 7.33 ± 5.71%. The correlation coefficient of sonographic volume or
size and histologic area was 0.71 (P = 0.11) and 0.79 (P = 0.32). The
relative blood volume within the tumor demonstrated sonographically
with the contrast agent correlated signficantly with histologic tumor
vascularity (r = 0.83, P < 0.001). Intraturmor vasularization/blood
volume was also demonstrated in the rabbit model.
Conclusions. There is a positive linear correlation between in vivo
sonographic tumor volume/size and histologic tumor size in our mouse
ocular melanoma model. Contrast-enhanced intensity corresponds with
microvascular density and blood volume in the model. High frequency
ultrasound with microbubble contrast enhancement is a real-time,
non-invasive and reliable method for in vivo evaluation of experimental
intraocular melanoma tumor area and relative blood volume.
Financial disclosure. Visual Sonices (TC)
21 RES 6
HISTOPATHOLOGICAL AND DOSIMETRIC FINDINGS
IN A PORCINE MODEL OF OCULAR RADIATION INJURY
FROM IODINE-125 PLAQUE BRACHYTHERAPY
Scott C. N. Oliver, MD, Victor Hsu, BA, Lucy Bollinger, Moyed Miften,
PhD, Laurie Gaspar, MD, Philip Boyer, MD (scott.oliver@ucdenver.edu)
Departments of Ophthalmology, Radiation Oncology and Pathology,
University of Colorado School of Medicine
Purpose. An animal model for acute radiation injury to the eye does
not exist. This study sought to describe the histopathological and
dosimetric findings in a porcine model of ocular radiation injury
from Iodine-125 (I-125) plaque brachytherapy.
Methods. The eyes from six pigs were irradiated with a high
dose of I-125 radiation (125-140 Gy), with progressively
lengthening survival durations of 1 to 11 weeks. Intraoperative
thermoluminescent dosimetry was compared to theoretical dose
calculations at five points along the globe. Sectioned eyes were
analyzed to determined histopathologic markers of acute radiation
injury.
Results. High-dose plaque brachytherapy in a porcine model was
technically feasible and well tolerated. Dosimetric measurements
demonstrated unacceptable variability and reproducibility when
oriented in a radial direction due to difficulty in positioning the
detectors on the posterior half of the globe. Equatorial orientation
of the dosimeters resulted in reliable measurements in close
agreement with theoretical dose calculations. Histopathological
evidence of acute radiation injury to the eye was detectable as

early as 2 weeks post irradiation, as evidenced by outer nuclear
layer (ONL) thinning, choroidal beading and thinning, and
derangement of retinal pigment epithelium (RPE) granules with
loss of RPE cells.
Conclusions. The porcine eye is a viable model for acute radiation
injury from I-125 plaque brachytherapy, with anatomical and size
similarity to the human eye. External dosimetry was validated
using an equatorial orientation of the detectors. For the first
time, histopathological markers of acute radiation injury were
identified, including ONL thinning of the retina, choroidal beading,
and RPE loss.
Financial disclosure. None
2347 RES 7
USE OF A MICROCATHETER TO OBTAIN BIOPSIES
FROM UVEAL MELANOMA
David J. Wilson1, MD; William Harbour2, MD (wilsonda@ohsu.edu)
1. Casey Eye Institute, Oregon Health and Science University
2. Barnes Eye Institute, Washington University
Purpose. To determine if a flexible microcatheter can be used to
obtain diagnostic material from uveal melanomas.
Methods. Following enucleation of two eyes with the clinical diagnosis
of choroidal melanoma, a flexible microcatheter (iScience) was
introduced into the supraciliary space. The catheter was advanced
posteriorly into the suprachoroidal space, with the progress of
the catheter monitored by direct observation of the blinking light
emitting diode at the tip of the catheter. When the catheter tip was
at the location of the uveal melanoma, cells were harvested from the
tumor using a specially designed trocar. Cells were analyzed using
routine cytology and gene expression profiling.
Results. Satisfactory material for cytologic diagnosis and gene
expression profiling was obtained for both eyes.
Conclusions. With further refinement, a flexible microcatheter might
permit sampling of posterior uveal melanomas to obtain clinically
useful information.
Financial disclosure. None
148 RES 8
EVALUATION OF HISTONE H3K4 METHYLATION BY
IMMUNOHISTOCHEMISTRY AND RELATIONSHIP TO
AGGRESSIVE UVEAL MELANOMA
L. Schoenfield1, M. Turell2, P. Carver1, R. Tubbs1, A. Singh2, Y.
Saunthararajah3 (schoenl@ccf.org)
Cleveland Clinic
1. Pathology and Lab Medicine Institute
2. Cole Eye Institute
3. Taussig Cancer Institute
Purpose. Aberrant histone modification patterns measured at the
level of single nuclei have been predictive of cancer recurrence and
poorer survival in multiple cancers. Similar evaluations have not been
performed in primary uveal melanoma (UM). Such evaluation could be
translationally useful, since unlike genetic alterations in UM, epigenetic
alterations might be reversible with chromatin-relaxing drugs.
RESEARCH DAY
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20
Methods. 23 cases of enucleated globes for UM from 2004-11 were
examined by immunohistochemistry for H3K4Me1 using a red chromagen
and compared to patient outcome as well as chromosome 3 or 3p26
status by FISH. Cases were interpreted as negative if at least 10% of the
nuclei had no staining.
Results. At the time of this study, 8 patients were DOD, 1 was alive with
metastasis, 2 were dead without evidence of metastasis and 12 were alive
without metastasis. Of the 13 negative cases, which would presumably
indicate a poorer outcome, 7 were dead of disease, 1 was dead without
metastasis (normal chromosome 3), and 5 were alive without metastasis
(all 5 with monosomy 3 or 3p26del). Of the 10 positive cases, 7 were
alive without metastasis, 1 alive with metastasis, 1 DOD, and 1 dead
without metastasis. When compared to monosomy 3 status alone, 10/13
negative cases and 4/10 positive cases had monosomy 3.
Conclusions. In this small study of 23 cases, lower global levels of
H3K4Me1 were significantly associated with death with metastases
(Chi-test p=0.02), suggesting an important role for therapeutically
targetable epigenetic alterations in UM pathogenesis.
Financial disclosure. None
42 RES 9
ROLE OF THE CHEMOKINE RECEPTOR CXCR4 IN
THE DEVELOPMENT AND PROGRESSION OF UVEAL
MELANOMA
Shahar Frenkel, MD, PhD1, Ariela Miller, MD1, Amnon Peled, PhD2, Jacob
Pe’er, MD1 (shahar.frenkel@gmail.com)
1. Departments of Ophthalmology and 2. Gene Therapy, Hadassah-
Hebrew University Medical Center, Jerusalem, Israel
Purpose. The purpose of this study was to determine the expression
of the CXCR4 chemokine receptor in primary and metastatic Uveal
Melanoma (UM) tissues and in a UM cell line. As well as the effect of
CXCR4 antagonist BKT-140 on two cell lines of UM.
Methods. Immunohistochemistry to detect CXCR4 (monoclonal antihuman
CXCR4, R&D systems, Minneapolis, USA) was performed on 17
eyes of 17 patients with UM, as well as on 9 liver sections of 9 metastatic
UM patients. The tissues were analyzed and graded according to the
intensity of the stain from 0-4 (0- being no staining, and 4- very intense
staining). The primary tumors were examined for tumor cell type,
vasculogenic mimicry patterns, mitotic figures, and tumor size (largest
basal diameter and tumor height). mRNA from OCM1 and C918 cell lines
were examined for the expression of CXCR4 before and after stimulation
with SDF1 (CXCL12), along with expression of SDF1 and VEGF mRNAs.
The expression of cell surface mRNA was examined in both cell lines. The
cell lines were also evaluated for the effect of BKT-140 CXCR4 receptor
agonist after 3hrs, 24hours and 48 hours at 10% Fetal Calf serum (FCS).
Results. CXCR4 expression was observed in both primary and metastatic
uveal melanoma. In the 17 primary tumors 10 (59%) tumors stained grade
3. The mean staining intensity was 2.59. In the metastatic tumor 3/9
(33.3%) liver sections stained grade 4. The mean staining intensity was
2.56. No correlation was found between CXCR4 staining intensity and
any of the histopathologic parameters. SDF1 mRNA was not detected
in OCM1 or C918 cell lines. mRNA levels of CXCR4 did not increase after
stimulation with SDF1, but this stimulation increased the mRNA levels
of VEGF after 24 and 48 hours in the OCM-1 cell line. BKT 140 caused
an increase in the number of dead cells after 3 hours of exposure but
this effect was less after 24 and 48 hours , a decrease in the number of
living cells was less significant in the OCM-1 lines at a dose of 100ug/
ml at 10% FCS after 3, 24 and 48 hours. In the C918 cell line there was

a reduction in the number of living cells especially at 48 hours and an
increase in the percent of dead cells at 10% FCS over 48 hours.
Conclusions. CXCR4 was found to be a relevant molecular pathway in
uveal melanoma as shown in the histopathologic sections from both
primary and metastatic tissues. Preliminary results for the use of the
inhibitor of this pathway, BKT-140, on cell lines show promise that this
inhibitor may have an additive effect in the treatment of UM.
Financial disclosure. Prof. Amnon Peled is the founder and CSO of Biokine Therapeutics Ltd.
The CXCR4 antagonist BKT140 belongs to Biokine Therapeutics.
1703 RES 10
EXPRESSION OF MACROPHAGE-ATTRACTION MOL-
ECULES IN UVEAL MELANOMA: INDUCTION BY HY-
POXIA?
I.H.G. Bronkhorst, M. Versluis, G.P.M. Luyten, P.A. van der Velden, M.J.
Jager (i.h.g.bronkhorst@lumc.nl)
Department of Ophthalmology, Leiden University Medical Center,
Leiden, The Netherlands
Purpose. Hypoxia and inflammation both play a role in cancer. In tumor
cells, hypoxia activates HIF-1α and NF-kB, and these factors may induce
a gene program that recruits leukocytes (through release of chemokines
and cytokines). This study was performed to examine whether hypoxia
induces uveal melanoma cells to release attractants for circulating
monocytes.
Methods. The inflammatory responses of four primary tumors were
studied in an in vitro 24 hour hypoxic culture system using quantitative
real-time PCR for expression of CSF-1, SDF-1,
MCP1 (CCL2), RANTES (CCL5), CCL7, CCL8, and VEGF.
Results. Inflammatory cytokine expression was variable in uveal
melanoma primary cultures. Under hypoxic conditions, we find an
upregulation of VEGF, and CCL7 mRNA, and a decrease of CSF-1 and
MCP1 mRNA. CCL8 was increased in two out of four cultures, and SDF-1
was not altered. CCL5 shows large intervariable differences, but was
only in one culture decreased.
Conclusions. Hypoxia influences inflammatory chemokine expression
in uveal melanoma cells. However, as the genomic levels of these
chemokines may not correlate directly with chemokine production, further
experiments with cell lines are needed. By negatively regulating adaptive
immunity, hypoxia may prevent excessive activation of the immune host
defense, which might otherwise lead to an anti-tumor response.
Financial disclosure. None
525 RES 11
ONCOGENE MUTATION PROFILING IN OCULAR
MELANOMA
Ivana K. Kim1, Laura MacConaill2, Emanuele Palescandolo2, Shan
Lu2, Joo-Eun Lee3, Scott Adams3, Margaret M. DeAngelis3, Paul Van
Hummelen2, Anthony Daniels1, Levi Garraway2, J. William Harbour4,
Evangelos S. Gragoudas1 (ivana_kim@meei.harvard.edu)
1. Massachusetts Eye and Ear Infirmary, Dept of Ophthalmology, Harvard
Medical School
2. Centre for Cancer Genome Discovery, Dana-Farber Cancer Institute,
Dept of Medical Oncology, Harvard Medical School, Boston, MA
3. Ocular Molecular Genetics Institute, Mass Eye and Ear Infirmary,
Harvard Medical School, Boston, MA
RESEARCH DAY
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21
4. Department of Ophthalmology and Visual Sciences, Washington
University School of Medicine, St. Louis, MO
Purpose. To perform a systematic screen in uveal melanoma samples for
known oncogenic mutations in order to identify potential therapeutic targets.
Methods. A high-throughput, mass-spectrometry based genotyping
technology (OncoMap) was employed to screen for over 1100 mutations
in 114 known cancer genes. DNA was purified from 146 specimens:
5 uveal melanoma cell lines, 87 fresh frozen (FF) samples of uveal
melanoma tissue, and 54 samples obtained from formalin-fixed,
paraffin-embedded (FFPE) tissue. After whole genome amplification,
adequate DNA was obtained from 128 of these samples for genotyping
with the OncoMap assay.
Results. Forty-two percent (42%) of samples passing all quality control
steps contained candidate mutations. Fifty-five candidate mutations
were found across 27 genes. Overall, only 25% of the mutation calls
were considered conservative based on the confidence of data review.
Validation studies using independent primers and unamplified DNA in
homogenous Mass-Extend (hME) assays confirmed only a few of the
candidate mutations. An FGFR3 mutation was validated in 3 samples
and the BRAF V600E mutation was validated in 3 uveal melanoma cell
lines. Eighty-eight percent of samples had GNAQ or GNA11 mutations.
Conclusions. This study confirmed the high frequency of GNAQ and
GNA11 mutations in uveal melanoma and demonstrated that uveal
melanoma appears to have a relative paucity of oncogenic mutations
seen in other tumour types.
Financial disclosure. None
1958 RES 12
DETECTION OF GNAQ/GNA11 MUTATIONS IN
CIRCULATING CELL-FREE DNA AS A BIOMARKER OF
UVEAL MELANOMA
Jordan Madic1,2, Bénédicte Trouiller1,2, David Gentien1,3, Maud
Milder1,4,5, Stéphanie Saada1,4,, Franck Assayag1,3,6, Fariba
Nemati1,3,6, Didier Decaudin1,3,6, Laurence Desjardins1,7, Sophie
Piperno-Neumann1,8, Olivier Lantz11,4,5,9, Marc-Henri Stern1,2 (jordan.
madic@curie.fr)
1. Institut Curie, Paris; 2. Inserm U830; 3. Département de Recherche
Translationnelle; 4. Département de Biologie des Tumeurs; 5. CIC-BT-
507 Inserm; 6. Laboratoire d’Investigation Préclinique; 7. Département
de Chirurgie; 8. Département d’Oncologie médicale; 9. Inserm U932.
Purpose. Circulating tumor-derived DNA (ctDNA) can be detected
in the plasma of cancer patients. Recently, ctDNA was proposed as a
biomarker to follow tumor burden and monitor treatment efficacy. However,
ctDNA represents a tiny fraction of the normal cell-free circulating DNA, and its
assessment is challenging due to specificity, sensitivity and quantitative issues.
We developed a real-time PCR based on the pyrophosphorolysisactivated
polymerization (bi-PAP) for the quantification of ctDNA in
plasma of patients with uveal melanoma (UM). Bi-PAP can detect known
point mutations independently of large excess of normal DNA. Our assay
targets the activating mutation of codon 626 of GNAQ or GNA11 which
are present in most UM.
Methods. Bi-PAP primer pairs specific for GNAQ 626A>T, GNAQ 626A>C
and GNA11 626A>T have been chosen. Their sensitivity and specificity
were assessed on serial dilutions of tumor DNA in normal DNA.
Results. Each assay could detect a single mutated molecule per reaction,
while 10,000 copies of normal DNA were not detected. In plasma of
mice bearing UM xenografts, ctDNA were proportional to the amount of
circulating human DNA and to the size of the xenograft. Furthermore, in

a retrospective analysis of 19 UM patient sera, 8 were found positive.
Conclusions. In conclusion, bi-PAP represents a promising tool for
the quantification of ctDNA in the blood of UM patients. A prospective
study is on going to confirm the clinical value of ctDNA level in metastatic
UM patients.
Financial disclosure. None
2109 RES 13
GNAQ/11 MUTANT DEPENDENT UVEAL MELANOMA -
SENSITIVITY TO MEK AND PI3K INHIBITION
Scott E. Woodman1A, Xiaoxing Yu1A, Jahan Khalili1A, Chandrani
Chattopadhyay1A, Michelle Williams1B, NancyPoindexter1A , Martine J. Jager2
, Elizabeth Grimm1A, Dan Gombos3, Bita Esmaeli3 (sewmdphd@yahoo.com)
1A. Melanoma Medical Oncology,
1B. Pathology, MD Anderson Cancer Center, Houston, TX;
2. Ophthalmology, Leiden University Med Center, Leiden, The Netherlands;
3. Head & Neck Surgery/Ophthalmology, MD Anderson Cancer Center,
Houston, TX.
Purpose. Metastatic uveal melanoma has a very poor prognosis and
no effective treatment. Activating GNAQ or GNA11 mutations have been
identified in approximately 85% of uveal melanoma tissues. The MEK/
MAPK and PI3K/AKT pathways may mediate GNAQ/11 growth and
survival signaling. The purpose of this study was to test the sensitivity
of uveal melanoma cell lines to combination treatment with clinically
relevant MEK and PI3K small molecule inhibitors.
Methods. The GNAQ/11 mutant (MUT) and wild-type (WT) status of
uveal melanoma cell lines was determined using standard sequencing
approaches. Concentration and time-course western blot experiments
were used to determine the optimal conditions for MEK inhibitor (MEKi)
or PI3K inhibitor (PI3Ki) treatment using GSK1120212 and GSK2126458,
respectively. Cell proliferation assays were performed to determine
the relative sensitivity of MUT vs. WT cells to MEKi and/or PI3Ki. Cell
cycle analysis was performed to determine the relative percentages
of cells in each stage before and after MEKi and/or PI3Ki treatment.
Annexin/Propidium-iodide staining was determined by flow cytometry
to assess the frequency of apoptotic cells before and after MEKi and/
or PI3Ki treatment. Reverse Phase Protein Array was employed to asses
downstream signaling changes that result from MEKi and/or PI3Ki treatment.
Results. Both MEKi and PI3Ki treatment result in early and sustained
loss of MAPK and AKT phosphorylation, respectively, at low nanomolar
concentrations in MUT and WT cells. MEKi and/or PI3Ki treatment
induces more pronounced G1 cell cycle arrest in MUT vs. WT cells.
However, neither MEKi nor PI3Ki treatment alone induces significant
apoptotic cell death in MUT or WT cells. The combination of MEKi +
PI3Ki also fails to induce apoptotic death in WT cells. Conversely,
the combination of MEKi + PI3Ki induces a marked increase (40-60%
of cells) in the subG0 cellular fraction of MUT cells consistent with
apoptotic death at 48 hrs. Annexin V staining is markedly increased
MUT cells after combination treatment. Cell signaling analysis
indicates that combined MEKi + PI3Ki treatment potentiates reduction
in 4EBP1-P, and increases in p27 and cleaved caspase 3 in MUT vs.
WT cells.
Conclusions. These results suggest that uveal melanoma cells with
activating mutations in GNAQ or GNA11 are highly dependent on
the MEK/MAPK and PI3K/AKT pathways for growth and survival.
Combination MEKi + PI3Ki small molecule treatment may be an
effective therapeutic strategy for the majority of metastatic uveal
melanoma patients.
Financial disclosure. GlaxoSmithKline
RESEARCH DAY
Abstracts
22
149 RES 14
INACTIVATING MUTATIONS IN BAP1 IN METASTASIZ-
ING CLASS 2 UVEAL MELANOMAS
J. William Harbour, Michael D. Onken, Lori A. Worley, Anne M. Bowcock,
Eli Roberson (harbour@vision.wustl.edu)
Washington University School of Medicine, St. Louis, Missouri, USA
Purpose. Uveal melanoma (UM), the most common primary cancer
of the eye, has a strong tendency for hematogenous metastasis.
UMs are grouped according to metastatic risk into class 1 (low risk)
and class 2 (high risk) based on gene expression profile. The class
2 signature is usually accompanied by monosomy 3, suggesting that
loss of chromosome 3 unmasks recessive mutations in a metastasis
suppressor gene (or genes) on the remaining copy of chromosome 3.
The purpose of this study was to identify this gene.
Methods. We performed exome capture and massively parallel DNA
sequencing in two class 2 tumors and identified a gene on chromosome
3 containing inactivating mutations in both tumors. Sanger sequencing
of the gene was then performed in additional class 1, class 2 and
metastatic tumors.
Results. Inactivating mutations were identified in BAP1, located at
chromosome 3p21.1, in 26/31 (84%) class 2 tumors 24/28 (86%), 2/3
(67%) liver metastases and in one atypical class 1 tumor that appeared
to be in transition to class 2. No mutations were found in 22 typical
class 1 tumors. The mutation was also present in normal blood DNA in
one case. RNA and protein levels were depleted in tumors harboring
mutations. RNAi mediated depletion of BAP1 in UM cells transformed
them from class 1 to class 2 phenotype.
Conclusions. This gene meets the criteria expected for a putative
metastasis suppressor gene on chromosome 3 in UM. This finding
provides novel opportunities for diagnostic testing and targeted
therapy.
Financial disclosure. Dr. Harbour and Washington University may receive remuneration in the
form of royalties based on a license of related technology by the University to Castle Biosciences,
Inc. This research was not funded by Castle Biosciences, Inc.
1944 RES 15
A RECURRENT DELETION OF THE BAP1 LOCUS IN
OCULAR MELANOMA
Suresh C. Jhanwar1, Yuqiang Fang1, Lu Wang1, Klaus Busam1, David H.
Abramson2 (Jhanwars@mskcc.org)
1. Department of Pathology, 2. Ophthalmic Oncology Service
Memorial Sloan-Kettering Cancer Center, New York, NY, 10065, USA
Purpose. A monosomy of chromosome 3 is one of the most common
cytogenetic abnormalities in ocular melanoma (OM), which may
be predictive of metastasis. Recent studies have shown that BAP1
gene located at 3p21.3 is mutated in approximately 84% of the
metastasizing OM.
Methods. We have performed FISH and SNP microarray analyses on
29 tumors to determine frequency and the nature of abnormalities
for chromosomes 1,3 and 8, in particular BAP1 locus on 3p21.3.
Results. FISH detected monosomy or a relative loss of the
chromosome 3p in 16/29 (55%); low to high level amplification of
MYC was detected in 7/21 (33%) and 14/21 (66%) respectively. The
abnormality of chromosome 1 included relative loss of 1p and gain of
1q in 7/29 (24%) of the cases. SNP arrays, on the other hand detected
hemizygous deletion of BAP1 gene in 18/20 (90%) of the cases, SNP-

array further detected copy number increase of MYC in 16/20 (80%),
whereas, deletion of 1p localized to a minimal region of deletion on
1p36.22 was seen in 13/20(65%) of the cases.
Conclusions. These results suggest that loss of BAP1 is the most
common genetic abnormality in OM, whereas deletion 1p36.22 &
MYC amplification may be secondary events. In addition, combined
use of these two methodologies is powerful to detect biologically
important abnormalities including copy number changes and
uniparental disomy. The clinical implications of these abnormalities,
specifically hemizygous deletion of BAP1 in relation to metastases,
prognosis and response to therapy are currently being examined.
Financial disclosure. None
1843 RES 16
BAP 1 MUTATION IN TWO FAMILIES WITH UVEAL
MELANOMA
Miguel A. Materin1, Camila Simoes1, Ruth Halaban1, William Harbour 2
(miguel.materin@yale.edu)
1. Yale School of Medicine, New Haven, Conncecticut
2. Washington University, St. Louis, Missouri
Purpose. To describe the potential inheritance of BAP 1 mutation in
patients with uveal melanoma.
Methods. Two different families with history of uveal melanoma
were found to have BAP1 mutation. Family 1, patient had choroidal
melanoma, class 2, BAP1 mutation. Maternal grandfather died
from liver metastases from an eye tumor. Patient’’s mother DNA
was positive for BAP1 mutation. Family 2, two brothers with uveal
melanoma had BAP1 mutation.
Results. We are presenting two different families with uveal
melanoma in two different members of the family and BAP1 mutation.
In one of the family, this mutation was found in a family member with
no uveal melanoma present.
Conclusions. We are presenting two different families with uveal
melanoma in two different members of the family and BAP1 mutation.
In one of the family, this mutation was found in a family member with
no uveal melanoma present.
Financial disclosure. Pfizer consultant: Miguel Materin, however, no commercial interest in this
paper.
103 RES 17
IDENTIFICATION OF MOLECULAR SUBGROUPS OF
RETINOBLASTOMA
M. Parulekar, G. Kapatai, M.A. Brundler, A. Peet, M. Wilson, H. Jenkinson,
C. McConville (manojparulekar@aol.com)
School of Cancer Sciences, University of Birmingham, Dept of
Ophthalmology, Oncology & Histopathology, Birmingham Children’’s
Hospital, UK
Purpose. Survival for retinoblastoma patients is excellent, but invasion
into the optic nerve or choroid is relatively frequent, increasing
the potential for extra-ocular metastasis. Little is known about the
molecular events which influence tumour behaviour. The purpose of
our research is to develop a clinically relevant molecular classification
of retinoblastoma and to translate this into 1H-MRS (1H-magnetic
resonance spectroscopy)-detectable markers for the non-invasive
RESEARCH DAY
Abstracts
23
diagnostic assessment of retinoblastomas.
Methods. Gene expression profiling was carried out on 21
retinoblastomas. Principal component analysis (PCA) was used for
classification of molecular sub-groups. Differentially expressed genes
in each subgroup were identified using SAM (Significance Analysis of
Microarrays). In vitro 1H-MRS was carried out to identify metabolite
spectra specific for molecular subgroups.
Results. Molecular analyses identified 3 distinct groups of
retinoblastomas characterized primarily by differing levels of
photoreceptor differentiation. One group showed expression of many
cone-associated genes, a second group expressed markers of rod,
cone and Müller glial cells, while the third showed down-regulation
of these genes.
Conclusions. Photoreceptor differentiation was inversely associated
with adverse histopathological features (choroid and post-laminar
optic nerve invasion), suggesting that loss of differentiation may
be associated with more aggressive tumour behaviour. Recurrent
chromosomal alterations characteristic of retinoblastoma (1q gain, 6p
gain, 16q loss) were almost entirely restricted to less differentiated
retinoblastomas indicating that genes on these chromosomes may
function in differentiation-related pathways and/or in the regulation of
cell cycle exit. Preliminary 1H-MRS results identified several metabolites
(e.g. glutamate/glutamine, taurine) which may have potential as
markers of retinoblastoma subgroups.
Financial disclosure. None
1820 RES 18
A NEW DISEASE: RETINOBLASTOMA WITH NO
DETECTABLE RB1 MUTATIONS DRIVEN BY MYCN
AMPLIFICATION
Brenda L. Gallie, D.E. Rushlow, S. Yee, J.Y. Kennett, P. Boutros, N.L. Prigoda-
Lee, W. Halliday, S. Pajovic, C. Spencer, B..LThériault, H. Dimaras, A. Raizis,
C. Houdayer, W.L. Lam, T. Corson, D. Lohmann (brenda@gallie.ca)
Retinoblastoma Solutions and the Toronto Western Hospital Research
Institute, University Health Network; Department of Molecular
Genetics, University of Toronto; British Columbia Cancer Research
Centre, Vancouver; The Ontario Institute for Cancer Research, Toronto;
Department of Pathology, Hospital for Sick Children, Toronto; Campbell
Family Cancer Research Institute and Ontario Cancer Institute, University
Health Network, Toronto; Departments of Hematology Oncology and
Ophthalmology and Visual Science, Hospital for Sick Children, Toronto;
Department of Molecular Pathology, Canterbury Health Laboratories,
Christchurch, New Zealand; Service de Génétique Oncologique, Institut
Curie and Université Paris Descartes, Paris, France; Eugene and Marilyn
Glick Eye Institute, Department of Ophthalmology; and Department of
Biochemistry and Molecular Biology, Indiana University School of Medicine,
Indianapolis, Indiana; Institut für Humangenetik, Universitätsklinikum
Essen, Germany; Dept. of Ophthalmology and Medical Biophysics, Univ
of Toronto, Canada.
Purpose. Dogma states that all retinoblastoma tumors have lost both
alleles of the RB1 tumor suppressor gene. We can efficiently identify
95% of RB1 mutant alleles. In 2% of tumors we failed to find any mutant
RB1 allele (RB1+/+).
Methods. In the RB1+/+ tumors, we characterized the RB and other
protein expression, the other genomic changes characteristic of primary
retinoblastoma, aCGH, and clinical features. One RB1+/+ cell line was
studied for growth rate in comparison to RB1-/- cell lines.
Results. In 1% of retinoblastoma tumors we discovered high level

amplification of the MYCN oncogene and normal RB1 (RB1+/+MYCNA
tumors). Array CGH showed that the genomic instability characteristic
of RB1-/- retinoblastoma was not present. The children with the
RB1+/+MYCNA tumors presented at very young ages (6 months vs 24
months for non-hereditary retinoblastoma). Distinctive histopathological
features included multiple nucleoli. In vitro the RB1+/+MYCNA cell line
rapidly died when MYCN levels are reduced by shRNA, in comparison to
RB1-/- cell lines with late acquired MYCN amplification that showed only
slowed growth when MYCN was reduced.
Conclusions
Through our clinical analysis of RB1 alleles in more than 1000
retinoblastoma probands, we have discovered a previously unrecognized
disease: retinoblastoma that is not caused by RB1 gene mutations. We
hypothesize that children presenting with unilateral retinoblastoma
filling the eye before 6 months of age, and those with extraocular/
metastatic retinoblastoma before 1 year of age, are likely to have
RB1+/+MYCNA tumors. Despite their rapid growth in very young children,
they may be more responsive to therapy than RB1-/- retinoblastoma,
since they have less genomic instability and therefore less capacity to
achieve drug resistance. Although RB1+/+MYCNA tumors are aggressive
and rapidly growing, targeting MYCN may cure.
Financial disclosure. D Rushlow, N Prigoda are employees of Retinoblastoma Solutions. Brenda
Gallie is the Medical Director and President of Retinoblastoma Solutions.
39 RES 19
TARGETING THE P-53 PATHWAY IN RETINOBLASTOMA
PRECLINICAL MODELS WITH SUBCONJUNCTIVAL
NUTLIN-3A
Rachel C. Brennan 1,2, Sara Federico1,2, Cori Bradley1, Jiakun Zhang1,
Jacqueline Flores-Otero1 , Matthew Wilson3, Clinton Stewart4, Fangyi
Zhu5, Kip Guy5, Michael A. Dyer1,3,6 (rachel.brennan@stjude.org)
1. Department of Developmental Neurobiology, St. Jude Children’s
Research Hospital; 2. Department of Hematology/Oncology, St.
Jude Children’s Research Hospital;3. Department of Ophthalmology,
University of Tennessee Health Sciences Center; 4. Department of
Pharmaceutical Sciences, St. Jude Children’s Research Hospital; 5.
Department of Chemical Biology and Therapeutics, St. Jude Children’s
Research Hospital, Memphis, USA; 6. Howard Hughes Medical Institute
Purpose. Virtually all human retinoblastomas express elevated
levels of MDM4 which silences the p53 pathway, thereby
providing an ideal molecular target for treating children with
advanced stage or refractory disease. A small molecule inhibitor
(nutlin-3a) of MDM2 also binds MDM4, but it does not efficiently
penetrate the blood-ocular barrier. This study tested the efficacy
of subconjunctival nutlin-3a in preclinical models of human
retinoblastoma (RB) and characterized the pharmacokinetics and
toxicity profile.
Methods. We developed an ocular formulation using FDA approved
adjuvants for ocular delivery. We performed pharmacokinetics of
subconjunctival nutlin-3a and compared the vitreal penetration to
that of systemic nutlin-3a. Comprehensive preclinical testing with
nutlin-3a (18 weeks) in two different genetic RB mouse models
and a human orthotopic xenograft were compared to the current
standard of care while monitoring ocular and systemic toxicity.
Results. Subconjunctival delivery of nutlin-3a gave 2,000-20,000
fold increase in intraocular penetration of drug compared to oral
or intravenous dosing. Using subconjunctival nutlin-3a once
every 3 weeks, we observed a dramatic response in our genetic
RESEARCH DAY
Abstracts
24
mouse model of RB with ectopic MDM4 expression. Importantly,
an identical model lacking p53 showed little response, providing
genetic validation of molecular targeting of MDM4 in RB. The
orthotopic xenograft showed a significant improvement in
outcome compared to current standard of care. Few ocular or
systemic toxicities were detected.
Conclusions. Subconjunctivally delivered nutlin-3a is a promising
new molecular targeted therapy for retinoblastoma. Our studies
provide a new standardized approach to evaluate novel agents for
incorporation into future clinical trials for retinoblastoma.
Financial disclosure. This work was supported by grants from the National Institutes of Health
(R01EY018599 and R01EY014867 (MAD) and CA23099 (CFS)); Cancer Center Support CA 21765
from the National Cancer Institute; and grants from the American Cancer Society, the Pew
Charitable Trust, and the American Lebanese Syrian Associated Charities (ALSAC). Dr. Dyer is a
Howard Hughes Medical Institute Early Career Investigator.
1656 RES 20
RB DEPLETION SELECTIVELY INDUCES PROLIFERATION
OF CONE-PRECURSOR LIKE CELLS
David Cobrinik, Xiaoliang L. Xu, Suresh C. Jhanwar, and David H.
Abramson (cobrinid@mskcc.org)
Department of Pediatrics, Department of Pathology, and Ophthalmic
Oncology Service, Memorial Sloan-Kettering Cancer Center, New York,
NY 10021, USA
Purpose. Biallelic inactivation of RB1 is thought to initiate
retinoblastoma tumorigenesis in a specific, yet undefined,
retinal cell type. We previously showed that retinoblastoma
cells prominently express markers of cones but not other cells,
and require factors (TRβ2 and RXRγ) and oncoproteins (N-Myc
and MDM2) that are especially prominent in post-mitotic cone
precursors. These observations are consistent with retinoblastomas
originating either from cone precursors or from cells that adopt
cone precursor circuitry after retinoblastoma inception. Here, we
explore whether cone-related features enable fetal retinal cell
proliferation in response to diminished Rb expression.
Methods. Rb was depleted in gestational week 18-21 retinal cells,
using lentiviral shRNA vectors, either with or without co-depletion
of cone-related factors or oncoproteins. The proliferative status
of cells prior to transduction was evaluated by EdU labeling, and
cellular phenotypes of proliferating cells were evaluated by costaining
for Ki67 and cell type-specific markers.
Results. RB1 knockdown induced proliferation of cells having
properties of cones but not other retinal cell types. Proliferating
cone-like cells lacked EdU, indicating that their forebears were
quiescent prior to Rb depletion. The proliferation of cone-like
cells was suppressed by co-depletion of TRβ2, MDM2, and N-Myc,
suggesting that these factors contribute to the proliferative
response to Rb loss.
Conclusions. Rb depletion provokes proliferation of fetal retinal
cells that have cone precursor properties and dependence
upon cone circuitry similar to retinoblastoma cells. Prospective
isolation of cells that proliferate in response to Rb depletion may
provide insight into the cell type that gives rise to retinoblastoma
tumors.
Financial disclosure. Supported by Perry’s Promise Fund, The Fund for Ophthalmic Knowledge,
Research and Development Funds of the MSKCC Department of Pathology, and NIH
R01CA137124.

615 RES 21
TRB2 AND SKP2 IN THE RETINOBLASTOMA PATH-
WAY
Xiaoliang L. Xu1,4, Renbing Jia1,5, Nengyi Zhou1, Xianqun Fan5, David H.
Abramson2, David Cobrinik ,4 and Suresh C. Jhanwar1 (xux2@mskcc.org)
1. Departments of Pathology; 2. Ophthalmic Oncology Service 3;
Department of Pediatrics
4. Sloan-Kettering Institute, Memorial Sloan-Kettering Cancer Center,
New York, USA; 5. Department of Ophthalmology, Shanghai Ninth
People’s Hospital, Shanghai Jiaotong University, Shanghai, P. R. China.
Purpose. We previously reported that TRB2 and SKP2 are essential
for retinoblastoma pathogenesis. In this study, we examine how TRB2
regulates SKP2.
Methods. We knocked down (KD) or overexpressed TRB1, TRB2, and
related genes in retinoblastoma and RB1 gene in colon cancer and
neuroblastoma to clarify the TRB and Rb signaling pathway.
Results. TRB2-KD promoted TRB1 activation, Emi1 inactivation, Cdh1
activation, and SKP2 degradation, resulting in S phase arrest. Conversely,
TRB1 KD promoted Emi1 activation, SKP2 stabilization, resulting in G2/M
block. TRB1 activated PP1-CDC25C-CDK1-PLK1 to phosphorylate and
inactivate Emi1, resulting in activation of Cdh1. TRB2 counteracted TRB1
and maintained PP2A mediated Emi1 dephosphorylation and activation.
The TRB knockout promoted, whereas TRB2 KO suppressed anterior
pituitary tumor formation in RB1+/- mice. Phospho-Rb binds to TRB2,
PP2A, and Emi1 to form a nuclear complex, named as S phase promoting
complex (SPC), which is essential for S-phase progression. RB1-KD in
neuroblastoma and colon cancer cells caused TRB1 activation, SPC
dissociation, Emi1 inactivation, Cdh1 activation, and SKP2 degradation,
resulting in S phase arrest.
Conclusions. G1-S and G2-M transitions exhibit a critical balance
regulated by TRB1 and TRB2, in which TRB2 promotes, whereas TRB1
suppresses G1/S transition. Following Rb mutation, TRB1 is activated,
resulting in SPC dissociation, Emi1 inactivation, cdh1 activation, and
SKP2 degradation preventing cell cycle reentry, which is a second
assurance for cell cycle control. Thus, high level TRB2 in cone precursors
counteracts TRB1 by maintaining SPC integrity, disrupting the doubleassurance,
and promoting cell cycle entry in retinoblastoma. The SPC
integrity may be an optimal therapeutic target for retinoblastoma.
Financial disclosure. Research and Development Funds of Department of Pathology, MSKCC.
The Fund for Ophthalmic Knowledge. Gerber Foundation.
1515 RES 22
NESTED RT-PCR DETERMINATION OF GD2 SYNTHASE
AS A MARKER FOR MINIMAL DISEASE IN RETINOBLAS-
TOMA IN THE CEREBRO-SPINAL FLUID (CSF)
Viviana Laurent, Claudia Sampor, Jorge Rossi, Mariano Gabri, Maria TG
de Davila, Daniel Alonso, Guillermo Chantada (gchantada@yahoo.com)
Hospital JP Garrahan, Universidad de Quilmes, Argentina
Purpose. The standard criteria for evaluation of the CSF in retinoblastoma
includes neuro-imaging and cerebrospinal fluid (CSF) cytology. We
hypothesized that RT-PCR-based techniques might increase the yield for
determining minimal dissemination and potentially guide CNS directed
therapy.
Methods. We evaluated the CSF of children with retinoblastoma who
presented IRSS stage 2-4 (n=14) and selected stage 1 with pathology risk
factors (n=8) at diagnosis, at 6 and 12 months and at relapse. Standard
evaluation included cell count and microscopical examination of the
RESEARCH DAY
Abstracts
25
cytoentrifugate. Minimal disease evaluation included immunocytology
for GD2 ganglioside and RT-PCR followed by nested-PCR amplification
of a GD2 synthase mRNA fragment.
Results. Three children underwent treatment for CNS invasion (2 trilateral)
diagnosed by standard evaluation. PCR was negative in both trilateral
cases and it was positive in the remaining case who had a persistent
positivity despite the CSF cytology cleared after chemotherapy. She had
a fatal CNS relapse thereafter. Eleven children with stage 2 to 4a disease
had no CNS involvement at diagnosis by standard evaluation and PCR
was positive in 4 (all of them had massive optic nerve involvement and 1
had also bone marrow metastasis). Two of them had a CNS relapse and
the remaining 2 are in complete remission for 9 and 18 months (followup
PCR is negative) after intensive therapy. CNS relapse occurred in 4
children with negative or not evaluable PCR (CNS mass with normal CSF
in 3). All children with stage 1 had negative CSF evaluation by all techniques
at all times and did not experience CNS relapse.
Conclusions. RT-PCR-based assays for the evaluation of the CSF status
in children with retinoblastoma may improve the sensitivity of standard
techniques by identifying minimal disease.
Financial disclosure. Supported by a grant from the Agencia de Promocion Cientifica y
Tecnologica, Ministerio de Ciencia, Tecnologia e Innovacion Productiva, Argentina, Fund for
Ophthalmic Knowledge, NYC, USA and Fundacion Natali Dafne Flexer, Buenos Aires, Argentina
1824 RES 23
SUPER-SELECTIVE INTRA-OPHTHALMIC ARTERY CHE-
MOTHERAPY: RETINAL ENDOTHELIAL TOXICITY IN
PRE-CLINICAL MODELS
Matthew W. Wilson, MD, FACS1,2,3, J. Scott Williams, MD, PhD4, John S.
Jackson, DVM5, Fan Wang, PhD6, Clinton F. Stewart, PharmD6, Timothy
D. Mandrell, DVM4, Barrett G. Haik, MD, FACS1,2, Jena J. Steinle, PhD1
(mwilson5@uthsc.edu)
University of Tennessee Health Science Center, 1. Hamilton Eye Institute,
Department of Ophthalmology; 4. Department of Radiology and 5.
Department of Comparative Medicine, Memphis, Tennessee, USA
St Jude Children’s Research Hospital, 2. Department of Surgery, Division
of Ophthalmology; 3. Department of Pathology; 6. Department of
Pharmaceutical Sciences, Memphis, Tennessee, USA
Purpose. To report in vitro and in vivo pre-clinical modeling of superselective
intra-ophthalmic artery chemotherapy (SSIOAC).
Methods. Cultured human retinal endothelial cells were exposed to
increasing concentration of melphalan and carboplatin. Post-exposure
cell death, proliferation and migration were measured. Surviving
cells were studied using microarray and ELISA. Six adult male Rhesus
macaques were randomly assigned to treatment with either 5 mg/30 mL
melphalan or 30 mg/30 mL carboplatin. Each animal underwent three
separate SSIOAC procedures at three-week intervals. Digital retinal
images were obtained during each infusion. Intravenous fluorescein
angiography was performed immediately after each procedure.
Results. Highest concentration of melphalan (4mg/ml) and carboplatin
(1mM) caused a 5 fold increase in cell death at 24 hours (p

artery precipitates were seen in 10 (of 18, 56%). Fluorescein angiogram
showed choroidal hypoperfusion (18 of 18, 100%). Histopathology
and electron microscopy showed retinal endothelial sloughing with
leukostasis, birefringent particles and vascular occlusion.
Conclusions. Both in vitro and in vivo models of SSIOAC showed marked
retinal endothelial cell toxicity with resultant vascular occlusion. Our
findings may explain the reported vascular toxicities following SSIOAC
in children.
Financial disclosure. None
2107 RES 24
EMBRYONIC RETINAL TUMORS IN TRANSGENIC MICE
CONTAIN CD133+ TUMOR-INITIATING CELLS
Richard L. Hurwitz1ABC,3 Lalita Wadhwa1,A, Wesley Bond1B, Laszlo
Perlaky1A, Paul Overbeek1, Mary Y. Hurwitz1AC, Patricia Chévez-
Barrios2,3 (rlhurwit@txch.org)
A Texas Children’s Cancer Center
B Translational Biology & Molecular Medicine
C Center for Cell & Gene Therapy
1 Baylor College of Medicine, Houston, TX
2 The Methodist Hospital, Houston, TX
3 Retinoblastoma Center of Houston, Houston, TX
Purpose. Human retinoblastomas caused by Rb1 mutations that result
in functionally defective or absent Rb1 protein originate during the
proliferative phase of retinal development. Similar retinal tumors occur
in mice only when multiple Rb family members are absent. Whether
the tumors originate from proliferating undifferentiated retinal cells or
from terminally differentiated retinal cells remains to be determined.
We tested the hypothesis that a tumor-initiating cell can be isolated from
tumors formed in developing, undifferentiated retinas of transgenic mice.
Methods. A transgenic mouse model of retinoblastoma was developed
by expressing SV40 T-antigen using the early eye development promoter
Pax6. T-antigen, which sequesters all Rb family members and p53, is
expressed in the retina and lens on E10 and tumors are observed by
E12.5, prior to retinal differentiation.
Results. A primary cell line developed from P7 murine tumors expresses
T-antigen, adheres in serum-containing media and forms neurosperes
in supplemented serum-free media. 1.5% of attached cells transferred
to serum-free medium form neurospheres. 0.5% of adherent cells are
CD133+. FACS sorted cells cultured in serum-free medium form 3-fold
more neurospheres in the CD133+ cultures than in the CD133- cultures
(p < 0.001). In vivo, 6/7 mice injected with CD133+ cells and 1/7 mice
injected with CD133- cells form tumors during the 6 month observation
period (p=0.007). These secondary tumors are indistinguishable from
the primary murine tumors.
Conclusions. There exist CD133+ cells within the developing, proliferating
murine retina that can initiate retinal tumors.
Financial disclosure. None
2333 RES 25
TREATMENT OF LHBETATAG RETINOBLASTOMA TU-
MORS WITH ANGIOGENIC AND GLYCOLYTIC INHIBI-
TORS AVOIDS CHEMOTHERAPY
Samuel K. Houston, Timothy G. Murray, Yolanda Pina, Christina Decatur
(shouston@med.miami.edu)
RESEARCH DAY
Abstracts
26
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA
Purpose. The purpose of this study was to evaluate the combination
treatment of angiogenic and glycolytic inhibitors on tumor burden and
hypoxia in advanced LHBETATAG retinoblastoma tumors.
Methods. Thirty advanced LHBETATAG mice (16 weeks of age) were
divided into 5 groups (n=6) and received injections of (a) saline, (b)
anecortave acetate (AA), (c) 2-deoxy-glucose (2-DG), (d) 2-DG 1-day
post-AA treatment), or (e) 2-DG 1-week post-AA treatment. Eyes were
enucleated at 21 weeks and tumor sections were analyzed for tumor
burden and hypoxia.
Results. Combined treatment with 2-DG and AA (2-DG 1-day and 1-week
post-AA) showed significant reduction in tumor burden compared to
saline treated eyes (61% and 56%, respectively) (P < 0.001). There
was not a significant effect of combination treatment when 2-DG was
injected 1-week post-AA when compared to 2-DG alone (P = 0.21).
However, eyes treated with 2-DG 1-day post-AA demonstrated a 23%
reduction in tumor burden compared with 2-DG alone (P = 0.03). In
addition, there was significant reduction in hypoxia with combined
treatment compared to saline controls (P < 0.001), with only 0.24%
and 0.3% hypoxia for 2-DG 1-day post-AA and 1-week post-AA,
respectively.
Conclusions. Combination therapy with angiogenic and glycolytic
inhibitors significantly enhanced tumor control and reduced tumor
hypoxia. There were synergistic effects of these two treatment
modalities that were dependent on treatment timing. Combination
treatment with angiogenic and glycolytic inhibitors avoided
chemotherapy in the treatment of LHBETATAG retinoblastoma tumors
and may potentially serve as adjuvant therapies in the treatment of
children with retinoblastoma.
Financial disclosure. None
50 RES 26
USING THE GLYCOLYTIC INHIBITOR 2-FLUORODEOXY-
D-GLUCOSE, A NOVEL APPROACH TO TARGET THE
CHEMORESISTANT CELL POPULATION IN LHBETATAG
RETINAL TUMORS
Yolanda Piña, Christina L. Decatur, Samuel Houston, Timothy G. Murray,
Ludimila Cavalcante, and Theodore Lampidis. (yoly26@yahoo.com)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA
Purpose. The aim of the current study is to assess the impact of
2-fluorodeoxy-D-glucose on tumor burden and hypoxia in advanced
LHBETATAG retinal tumors.
Methods. The study protocol was approved by the University of Miami
Institutional Animal Care. 17-week-old (n=54) LHBETATAG transgenic
mice were treated with 2-fluorodeoxy-D-glucose or saline control.
These animals received three different treatments. They were treated:
(1) with one injection and sacrificed at one day post-treatment, (2) with
one injection and sacrificed at one week post-treatment, or (3) twice a
week for three weeks and sacrificed at one day post-last injection. At
the time of enucleation, all eye samples were snap frozen and analyzed
for tumor burden and hypoxia using immunohistochemical techniques.
Average densities of the different groups were statistically analyzed
using ANOVA. Results were considered significant if p≤ 0.05.
Results. There was no apparent toxicity associated with 2-fluorodeoxy-
D-glucose treatment. There was a significant reduction in tumor burden

following treatment with 2-fluorodeoxy-D-glucose at 1 day (86%)
and 3 weeks (63%) post-treatment (p≤0.05). There was no reduction
of tumor burden observed when mice were treated with 1 injection
and eyes harvested at 1 week post-treament (2%, p=0.0640). There
was a significant reduction of hypoxia areas following treatment with
2-fluorodeoxy-D-glucose at 1 day (100%) and 3 weeks (75%) posttreatment
(p≤0.05). There was an increase in hypoxia of 12% following
treatment at 1 week post-injection, but this increase was not statistically
significant.
Conclusions. 2-FDG significantly reduces tumor burden and tumor
hypoxia following a single injection, with continued efficacy following
repeated injections for 3 weeks. 2-FDG treatment is efficacious in
murine retinoblastoma tumors and may enhance tumor control when
combined with other therapies. 2-FDG appears to target hypoxic cells,
a population that has been resistant to chemotherapy and radiation.
Additionally, 2-FDG is commonly used in medical imaging and does not
pose significant toxicities.
Financial disclosure
This work was supported by the American Cancer Society and the
University of Miami Sylvester Comprehensive Cancer Center. NIH center
grant P30EY014801 and by an unrestricted grant to the University of
Miami from Research to Prevent Blindness.
2140 Res 27
MTOR TARGETING IN COMBINATION WITH CHEMO-
THERAPY: UPSTREAM REGULATION OF ANAEROBIC
GLYCOLYSIS TO TARGET THE CHEMORESISTANT CELL
POPULATION IN RB
Timothy G. Murray, Yolanda Piña, Christina L. Decatur, Samuel Houston,
Ludimila Cavalcante, and Theodore Lampidis (tmurray@med.miami.edu)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA.
Purpose. To assess the efficacy of the combination therapy utilizing
carboplatin chemotherapy and rapamycin mammalian target of
RESEARCH DAY
Abstracts
27
rapamycin (mTOR) inhibitor on tumor burden, hypoxia, and blood
vessels in the LHBETATAG transgenic mouse model of retinoblastoma.
Methods. The study protocol was approved by the University of
Miami Institutional Animal Care and Use Review Board Committee. 12week-old
(n=62) LHBETATAG transgenic mice were treated with the
chemotherapeutic agent carboplatin, the mTOR inhibitor rapamycin, or
both. Animals were treated with subconjunctival injections twice a week
and received treatment for either 1 week or 3 weeks. All animals were
sacrificed at 1 day post-last injection and eyes were enucleated. At the
time of enucleation, all eye samples were snap frozen and analyzed for
tumor burden, hypoxia, new blood vessels, and mature blood vessels
using histopathology and immunohistochemistry. Data were statistically
analyzed using ANOVA.
Results. Mice that received treatment for 1 week had a tumor reduction
of 23.8%, 49.3%, and 60.6% when treated with carboplatin, rapamycin,
and carboplatin + rapamycin, respectively (p

Morning
8.30-9.20 Poster presentations Rbp100-120
(moderators C. Shields, P. Chevez-Barrios)
9.20-10.25 Papers
(moderators A.C. Moll, M.W. Wilson)
152 RB1
GUIDELINES FOR IMAGING OF RETINOBLASTOMA:
DIAGNOSTIC IMAGING STRATEGY AND STANDARD-
IZED MR IMAGING PROTOCOL
P. de Graaf, S. Göricke , F.Rodjan , P. Galluzzi, P. Maeder, J.A. Castelijns,
H.J. Brisse on behalf of the European Retinoblastoma Imaging
Collaboration (ERIC)
1831 RB2
CHEMOTHERAPY FOCAL THERAPY AND BEYOND:
THE IMPORTANCE OF CLINICAL TRIALS & THE ROLE OF
A RETINOBLASTOMA CLINICAL STUDIES CONGLOMER-
ATE.
Helen Dimaras, Ashwin C. Mallipatna, Brenda L. Gallie, Helen S.L. Chan.
1846 RB3
RETINOBLASTOMA: ANALYSIS OF MORTALITY IN UZ-
BEKISTAN
Z. Islamov, F. Islom
1201 RB4
INCIDENCE OF RETINOBLASTOMA IN THE NETHER-
LANDS 1950 - 2010: A SHIFT IN THE PROPORTION OF
HEREDITARY RETINOBLASTOMA.
M.I. Bosscha, L. Razzaq, C.J. Dommering, F.E. van Leeuwen, A.C. Moll
1844 RB5
EARLY DELIVERY OF INFANTS FOR TREATMENT OF
RETINOBLASTOMA DIAGNOSED BY PRENATAL RB1
MUTATION IDENTIFICATION
Brenda L. Gallie, Helen Dimaras, Elise Héon, Joanne Sutherland, Megan
Day, Vikas Khetan, Gardiner Jane, Helen Chan
43 RB6
CLINICAL AND GENETIC FEATURES OF REGRESSED
RETINOBLASTOMA
Manoj Parulekar, Archana Kulkarni, Trevor Cole, Bruce Morland, Helen
Jenkinson, Sarah Turner, John Ainsworth
47 RB7
RETINOBLASTOMA PATIENTS WITH CHROMOSOME
13Q DELETIONS HAVE INCREASED CHEMOTHERAPY-
RELATED TOXICITIES
R.C. Brennan, I. Qaddoumi, C. Billups, C. Odom, T. Douglas, W. Furman, M.W. Wilson
Tuesday November 15, 2011
RETINOBLASTOMA
Program
29
10.20-10.50 BREAK
10.50-12.25 Papers
(moderators L. Desjardins, L. Teixeira)
358 RB8
TOPOTECAN IS EFFECTIVE IN ADVANCED INTRAOCU-
LAR RETINOBLASTOMA WITH MANAGEABLE TOXIC-
ITY
Ibrahim Qaddoumi, Catherine Billups, Clinton F. Stewart, Jianrong Wu,
Katherine Helton, Mary McCarville, Thomas E. Merchant, Rachel Brennan,
Barrett G. Haik, Carlos Rodriguez-Galindo, Matthew W. Wilson
619 RB9
LACK OF ACUTE TOXICITY FOLLOWING STANDARD
DOSE CARBOPLATIN, ETOPOSIDE AND VINCRISTINE:
RESULTS FROM CHILDREN’S ONCOLOGY GROUP
STUDY ARET-0332
A. Leahey, P. Chevez-Barrios, B. Langholz, A. Javed, V. Khetan, S. Honavar,
R. Eagle, D. Albert J. O’Brien, K. Matthay, A. Meadows, M. Chintagumpala
1615 RB10
CONSERVATIVE TREATMENT OF INTRAOCULAR RETIN-
OBLASTOMA: A PROSPECTIVE PHASE II RANDOMIZED
TRIAL OF NEOADJUVANT CHEMOTHERAPY FOLLOWED
BY LOCAL TREATMENT
Livia Lumbroso-Le Rouic, Isabelle Aerts, David Hajaje, Christine Lévy-
Gabriel, Alexia Savignoni, Nathalie Algret, François Doz, Laurence
Desjardins
24 RB11
OUTCOMES OF 105 EYES WITH INTRAOCULAR RETIN-
OBLASTOMA TREATED WITH SYSTEMIC CHEMOTHER-
APY AND/OR LOCAL THERAPY AS FIRST TREATMENT
Luiz F. Teixeira, Carla R. Donato Macedo, Virginia L. Torres, Camila H.
Hashimoto, Juliana dos Santos Soares, Clelia M. Erwenne
1546 RB12
BRACHYTHERAPY IN THE TREATMENT OF RETINO-
BLASTOMA. EXPERIENCE IN A SPANISH POPULATION
Mónica Asencio-Duran, José Abelairas, José-María Peralta, José-Maria
Fernández-Guardiola, Ernesto Sánchez-jacob,
2130 RB13
RADIOTHERAPY IN COMPLEX TREATMENT OF AD-
VANCED RETINOBLASTOMA IN CHILDREN
Tatiana Ushakova, Igor Glekov, Olga Gopovtzova, Igor Dolgopolov,
Alentina Pavlovskaya, Irina Matveeva, Vladimir Polyakov, Geordge
Mentkevich

1909 Rb14
RESULTS OF PATIENTS WITH UNILATERAL RETINO-
BLASTOMA TREATED WITH INITIAL ENUCLEATION: A
SINGLE INSTITUTION IN BRAZIL
Carla R. Donato Macedo, Luiz F. Teixeira, Camila H Hashimoro, Virginia L.
L Torres, Juliana dos Santos Soares, , Maria T. Seixas, Maria C. Martins,
Clelia M. Erwenne
1940 RB15
PROTON THERAPY FOR RECURRENT LOCALLY AD-
VANCED RETINOBLASTOMA: COMBINED RESULTS
FROM THE RETINOBLASTOMA CENTER OF HOUSTON
& INDIANA UNIVERSITY
D.S. Gombos, A.L. Chang, D.L. Andolino, H.P. Fontanilla, C. Herzog, M.
Chintagumpala, P. Zage, R. Hurwitz, P. Chevez-Barrios, A. Mahajan
1828 RB16
COMPARISON OF HIGH-RISK HISTOPATHOLOGY
BETWEEN UNTREATED AND TREATED EYES OF
PATIENTS WITH RETINOBLASTOMA
M.W. Wilson, R. Brennan, C. Rodriguez-Galindo, C. Billups, B.G. Haik, I.
Qaddoumi
2 Rb17
PATHOLOGY ASSESSMENT IN UNILATERAL RETINO-
BLASTOMA WITH & WITHOUT HISTOPATHOLOGIC
HIGH-RISK FEATURES & THE ROLE OF ADJUVANT
CHEMOTHERAPY: A COG STUDY
P. Chévez-Barrios, R. Eagle, D. Albert, G. Vemuganti, S. Krishnakumar,
B. Langholz, S. Honavar, J. O’Brien, A. Leahey, K. Mattha , A. Meadows,
M. Chintagumpala
12.25-12.50
Keynote Lecture: Dr. Francis L. Munier
CURRENT APPROACHES IN TREATING RETINOBLAS-
TOMA
12.50-14.10 LUNCH
14.10-14.50 Posters presentations Rbp121-138
(Moderators D.H. Abramson, M.A. Reddy)
14.50-16.10 Papers
(moderators J. Hungerford, A. Fandiño)
RETINOBLASTOMA
Program
30
2123 RB18
LESSONS LEARNED AFTER 5 YEARS (AND 500
INFUSIONS) OF CHEMOSURGERY: PREDICTORS OF
SUCCESS AND FAILURE
David H. Abramson, Brian P. Marr, Scott E. Brodie, Ira J. Dunkel, Y. Sotiria
Palioura, Pierre Gobin
147 RB19
SUPER SELECTIVE OPHTHALMIC ARTERY INFUSION
OF CHEMOTHERAPYFOR INTRAOCULAR RETINO-
BLASTOMA TREATMENT: INITIAL EXPERIENCE IN AR-
GENTINA
Adriana C. Fandiño, Alejandro Ceciliano, Guillermo L. Chantada,
Francisco Villasante, Eduardo Lagomarsino, Julieta Lia Domínguez,
Claudia Sampor, Paula Schaiquevich, José Lipsich, Julio E. Manzitti
2348 RB20
INTRA-ARTERIAL CHEMOTHERAPY FOR RETINOBLAS-
TOMA: EXPOSURES, TUMOR CONTROL, COMPLICA-
TIONS - NEW OBSERVATIONS.
Carol L. Shields., Swathi Kaliki, Sanket U. Shah, Carlos G. Bianciotto,
Jerry A. Shields.
1527 RB21
COMPARATIVE PHARMACOKINETICS OF TOPOTECAN
AND MELPHALAN AFTER INTRA-ARTERIAL ADMINIS-
TRATIONS IN THE SWINE MODEL
Paula Schaiquevich, Emiliano Buitrago, Ana Torbidoni, Alejandro
Ceciliano, Adriana Fandino, Javier Opezzo, Marcelo Asprea, Sergio
Sierre, Flavio Requejo, David H. Abramson, Guillermo F. Bramuglia,
Guillermo L. Chantada.
539 RB22
RELAPSES FOLLOWING INTRA-ARTERIAL CHEMO-
THERAPY WITH MELPHALAN
T. Hadjistilianou, S. De Francesco, S. Bracco, P. Galluzzi, P. Toti, P.
Gennari, A. D’Ambrosio, M. Caini, D. Galimberti, A. Cerase, C. Venturi
2257 RB23
FACTORS INFLUENCING RESPONSE RATE FOLLOWING
INTRA-OPHTHALMIC ARTERY MELPHALAN SALVAGE
THERAPY FOR RELAPSE AFTER PRIMARY TREATMENT
OF RETINOBLASTOMA
John Hungerford, Judith Kingston, Stefan Brew, Ashwin Reddy, Mandeep
Sagoo, Fergus Robertson, Jane Herod
60 RB24
INTRAVITREAL CHEMOTHERAPY WITH MELPHALAN FOR
VITREOUS DISEASE IN ADVANCED RETINOBLASTOMA:
EVIDENCE FOR SAFETY AND EFFICACY
Francis L. Munier, Marie-Claire Gaillard, Aubin Balmer, Susan Houghton,
Maja Beck-Popovic

7 RB25
SELECTIVE INTRA-ARTERIAL CHEMOTHERAPY AS A
TREATMENT FOR INTRAOCULAR RETINOBLASTOMA:
ALTERNATIVES TO DIRECT OPHTHALMIC ARTERY
CANNULATION
Michael A. Klufas, Y. Pierre Gobin, Brian Marr, Scott E. Brodie, Ira J.
Dunkel, David H. Abramson
1448 RB26
INTRA-ARTERIAL CHEMOTHERAPY USING SIMUL-
TANEOUS MULTI AGENT CHEMOTHERAPY, THREE
DRUGS, FOR RESCUE OF EYES WITH INTRAOCULAR
RETINOBLASTOMA.
Brian P. Marr, Y. Pierre Gobin, Scott E. Brodie, Ira J. Dunkel, David H.
Abramson
1550 RB27
OCULAR COMPLICATIONS OF DIRECT INTRA-OPH-
THALMIC ARTERY MELPHALAN TREATMENT FOR RE-
FRACTORY RETINOBLASTOMA
M Ashwin Reddy, Wisam J. Muen, Judith Kingston, John Hungerford,
Fergus Robertson, Stefan Brew, Mandeep Sagoo, Dorothy Thompson
16.00-16.20 BREAK
16.20-17.20 Papers
(moderators T. Murray, T. Hadjistilianou)
438 RB28
LIMITATIONS OF THE INTERNATIONAL CLASSIFICA-
TION IN PREDICTING SUCCESS OF INTRA-ARTERIAL
CHEMOTHERAPY FOR GROUP D/E INTRAOCULAR
RETINOBLASTOMA
Sotiria Palioura, Y. Pierre Gobin, Scott E. Brodie, Brian P. Marr, Ira J.
Dunkel, and David H. Abramson
2330 RB29
INTRA-ARTERIAL MELPHALAN DOSING REGIMENS
FOR THE TREATMENT OF RETINOBLASTOMA
Timothy G. Murray, Samuel K. Houston, Mohammad A. Aziz-Sultan,
Christina E. Fernandes, Christina Decataur, Yolanda Pina
15 RB30
THE GENERAL HEALTH AND PSYCHOSOCIAL FUNCTIONING
OF ADULT SURVIVORS OF RETINOBLASTOMA:PRELIMINARY
RESULTS OF THE RETINOBLASTOMA SURVIVOR STUDY
Ira J. Dunkel, Jennifer S. Ford, Charles A. Sklar, Kevin C. Oeffinger, Joanne F.
Chou, Yuelin Li, Danielle Novetsky Friedman, Mary McCabe, Nancy Kline,
Leslie L. Robison, Ruth A. Kleinerman , Brian P. Marr, David H. Abramson
RETINOBLASTOMA
Program
31
2323 RB31
LATE MEDICAL OUTCOMES IN SURVIVORS OF EXTRA-
OCULAR RETINOBLASTOMA: THE MEMORIAL SLOAN-
KETTERING CANCER CENTER (MSKCC) EXPERIENCE
D. Novetsky-Friedman, C.A. Sklar, K.C. Oeffinger, N.A. Kernan Y. Khakoo,
B.P. Marr, S.L. Wolden, D.H. Abramson, I.J. Dunkel
1755 RB32
VARIATION OF SECOND CANCER RISK BY FAMILY
HISTORY OF RETINOBLASTOMA AMONG LONG-TERM
SURVIVORS
Ruth A. Kleinerman, Chu-ling Yu, Mark P. Little, Yi Li , David H. Abramson,
Johanna H. Seddon, and Margaret A. Tucker
2039 RB33
A NOVEL, ORAL, NON-INVASIVE METHOD OF DELIV-
ERY FOR THE GLYCOLYTIC INHIBITOR 2-DEOXY-D-
GLUCOSE IN THE TREATMENT OF RETINOBLASTO-
MA.
Christina L. Decatur, Yolanda Piña, Samuel Houston, Ludimila Cavalcante,
Theodore Lampidis, Timothy G. Murray
1352 RB34
VITREOUS INJECTION THERAPY OF MELPHALAN FOR
RETINOBLASTOMA
Shigenobu Suzuki, Akihiro Kaneko
58 RB35
A STUDY OF UNILATERAL RETINOBLASTOMA WITH
AND WITHOUT HISTOPATHOLOGIC HIGH-RISK FEA-
TURES AND THE ROLE OF ADJUVANT CHEMOTHERA-
PY: A CHILDREN’S ONCOLOGY STUDY
M. Chintagumpala, R. Eagle, D. Albert, B. Langholz, V.A. Reddy, V.
Khetan, S. Honavar S, J. O’Brien, A. Leahey, K. Matthay, A. Meadows, P.
Chevez-Barrios
17.20-18.20 ISOO Business meeting

Posters Retinoblastoma
2006 RBp100
RETINOBLASTOMA ASSESSMENT BY DOPPLER SONOG-
RAPHY - A FOLLOW-UP STUDY
Maria Teresa B.C. Bonanomi, Osmar C. Saito, Tatiana Tanaka
354 RBp101
PATIENTS WITH UNILATERAL RETINOBLASTOMA AND
HIGH-RISK PATHOLOGIC FEATURES DO NOT REQUIRE
INTENSIVE METASTATIC WORK-UP OR AGGRESSIVE
CHEMOTHERAPY
Ibrahim Qaddoumi, Matthew W. Wilson, Catherine Billups, Jianrong
Wu, Thomas Merchant, Barry Shulkin, Rachel Brennan, Barrett G. Haik,
Carlos Rodriguez-Galindo, Erin Sullivan.
1916 RBp103
TEN YEARS OF EXPERIENCE IN THE TREATMENT OF
INTRA-OCULAR RETINOBLASTOMA IN A SINGLE IN-
STITUTION IN BRAZIL
Carla R.D. Macedo, Luiz F. Teixeira, Camila H. Hashimoto, Virginia L.
Torres, Juliana dos Santos Soares, Maria T. Seixas, Maria C. Martins,
Clelia M. Erwenne
60 RBp104
DIFFERENT CHARACTERISTICS OF GERMLINE AND
NON-GERMLINE RETINOBLASTOMA
Fariba Ghassemi, Hormoz Chams, Siamak Sabour, Reza Karkhaneh,
Farzad Farzbod, Mehdi Khodaparast, Parvaneh Vosough
1833 RBp105
RETINOBLASTOMA IN CHILDREN: ANALYSIS OF 518 CASES
Z. Islamov, F. Islom
102 RBp106
MULTIDISCIPLINARY COORDINATED SERVICES:
WHAT WE NEED TO IMPROVE IN THE MANAGEMENT
OF RB
M Naseripour, H Nazari, KH Ghasemi Falavarjani, R Soudi, A Maleki, S
Ansari,
40 RBp107
EXPERIENCE IN TREATMENT OF METASTATIC & NON-
METASTATIC ORBITAL RETINOBLASTOMA IN A SIN-
GLE INSTITUTION
Carlos A. Leal, Julieta Robles-Castro, Gabriela Isaac, Vanessa Bosch
836 RBp108
ERG MONITORING OF RETINAL FUNCTION DURING
SYSTEMIC CHEMOTHERAPY FOR RETINOBLASTOMA.
Scott E. Brodie, Yannis M. Paulus, Mrinali Patel, Y. Pierre Gobin, Ira J.
Dunkel, Brian Marr, David H. Abramson
RETINOBLASTOMA
Posters
32
2117 RBp109
UTERINE LEIOMYOSARCOMA IN RETINOBLASTOMA:
HOW SHOULD WE COUNSEL OUR PATIENTS?
Jasmine H. Francis, Ruth A. Kleinerman, David H. Abramson
1700 RBp110
MONITORING AND DISCUSSING QUALITY OF LIFE IN
RETINOBLASTOMA ROUTINE PRACTICE
A.C. Moll, M.I. Bosscha, G. LaRiviere, W.A. Kors, I. Verdonck-de Leeuw,
J. van Dijk, J. Huisman
242 RBp111
DOES PARENTAL SOCIOECONOMIC AND EDUCA-
TIONAL STATUS INFLUENCE RETINOBLASTOMA INI-
TIAL PRESENTATION AND ITS SUBSEQUENT MOR-
BIDITIES?
Daniel Colicchio, Carla D. Macedo, Juliana dos Santos Soares, Luiz F.
Teixeira
1534 RBp112
ARE SOCIOECONOMIC STATUS AND ETHNICITY RISK
FACTORS FOR THE PRESENTATION OF ADVANCED RB
IN THE UK?
M. Ashwin Reddy, Rabia Bourkiza, Archana Kulkarni, Manoj Parulekar,
Phillippa Cumberland, Mandeep Sagoo, John Hungerford, Jugnoo Rahi
352 RBp113
NEUROPSYCHOMOTOR DEVELOPMENT OF EYE
ENUCLEATED CHILDREN WITH RETINOBLASTOMA
Marcela Bagnatori Braga, Juliana dos Santos Soares, Carla R.D. Macedo,
Walquyria de Almeida Santos
345 RBp114
NURSING GUIDELINES FOR SELF-CARE OF PATIENTS
WITH PROSTHETIC RETINOBLASTOMA: AN EXPERI-
ENCE REPORT
Juliana dos Santos Soares, Carla R.D. Macedo, Adriana M. Duarte
33 RBp115
EYE SALVAGE RATE IN 166 PATIENTS WITH
INTRAOCULAR RETINOBLASTOMA
Luiz F. Teixeira, Carla R.D. Macedo, Virginia L. Torres, Camila H.
Hashimoto, Rubens Belfort Neto, Juliana dos Santos Soares, Clelia
Maria Erwenne
933 RBp116
SYSTEMIC CHEMOTHERAPY IN THE MANAGEMENT
OF RETINOBLASTOMA
John D McKenzie, Nisha Sachdev, Sandra Staffieri, Kary Suen, James E
Elder, John Heath, Peter Downie

2225 RBp117
COMPARISON BETWEEN OPHTHALMIC ARTERIAL IN-
JECTION THERAPY AND CHEMOREDUCTION AS PRIMA-
RY THERAPY FOR INTRAOCULAR RETINOBLASTOMA
Takashi Yamane, Nobuyuki Suzuki, Makoto Mohri
942 RBp118
INTRA-ARTERIAL CHEMOTHERAPY FOR RETINOBLAS-
TOMA: FIRST AUSTRALIAN EXPERIENCE
John D McKenzie, Nisha Sachdev, Sandra Staffieri, James E Elder, John
Heath, Peter Downie, Peter J Mitchell
817 RBp119
OUTCOMES OF GROUP D RETINOBLASTOMA EYES
J.L. Berry, H. Almarzouki, S.R. Bababeygy, T.H. Lee, R. Jubran, A.L. Murphree
417 RBp120
THE FIRST EXPERIENCE OF RETINOBLASTOMA
TREATMENT WITH THE USE OF SUPERSELECTIVE
INTRA-ARTERIAL CHEMOTHERAPY IN RUSSIA
S.V. Saakyan, S.B. Yakovlev, G.L. Kobyakov, N.K. Serova, A. Jarwa
2317 RBp121
MISSING INTRAOCULAR BLUSH IN AN ANGIOGRAPHY
OF AN ADVANCED INTRAOCULAR RETINOBLASTOMA
M. Holdt, S. Göricke, E. Biewald, M. Schündeln, N. Bornfeld, M.
Schlamann
2004 RBp122
CONSERVATIVE TREATMENT OF INTRAOCULAR
RETINOBLASTOMA: A PROSPECTIVE PHASE II TRIAL
FOR BILATERAL RETINOBLASTOMA WITH MACULAR
OR PARAMACULAR INVOLVEMENT
Christine Levy-Gabriel, Livia Lumbroso-Le Rouic, Isabelle Aerts, David
Hajaje, Alexia Savignoni, Nathalie Algret, François Doz, Laurence Desjardins
1857 RBp123
EXTRACTION OF RADIATION-INDUCED CATARACT IN
PATIENTS WITH RETINOBLASTOMA
Z. Islamov, F. Islom
1533 RBp124
OUTCOME OF CHILDREN WITH RETINOBLASTOMA
AND ISOLATED CHOROIDAL INVASION
Andrea Bosaleh, Claudia Sampor, Verónica Solernou, Adriana Fandiño,
Julieta Domínguez, María TG de Dávila, Guillermo Chantada
2336 RBp125
HIGH RISK HISTOPATHOLOGY FOLLOWING INTRA-
ARTERIAL (IA) CHEMOTHERAPY FOR RETINOBLAS-
TOMA
D. Gombos, C. Shields, J. Shields, P. Chevez Barrios, R. Eagle, C. Herzog,
M. Chintagumpala, R. Hurwitz, P. Zage, R. Spencer
RETINOBLASTOMA
Program
33
1613 RBp126
DIFFUSE INFILTRATING RETINOBLASTOMA AFTER
CHEMOREDUCTION FAILURE
Hans E. Grossniklaus, Christopher K. Hwang, Thomas M. Aaberg,
Jr., Patricia Chevez-Barrios, Dan Gombos, Evelyn Paysse, Murali
Chintagumpala, Elizabeth Verner-Cole
30 RBp127
TREATMENT MODULATION IN RETINOBLASTOMA TU-
MORIGENESIS AND ITS IMPACT ON TUMOR BURDEN
Timothy G. Murray, Samuel Houston, Christina L. Decatur, Nikesh Shah,
Ludimila Cavalcante, and Yolanda Piña
1603 RBp128
REPORT OF NINE NOVEL RB1 MUTATIONS IN
PATIENTS WITH RETINOBLASTOMA REFERRED TO
RASOUL-E-AKRAM UNIVERSITY HOSPITAL
Babak Behnam, Ali Ahani, Masoud Naseripour
1954 RBp129
A NOVEL GERMLINE MUTATION OF THE RETINOBLAS-
TOMA SUSCEPTIBILITY GENE IS ASSOCIATED WITH
INCOMPLETE PENETRANCE
Peter Hovland, Kami Schneider, Edythe Albano
601 RBp130
CNS ABNORMALITIES IN RTB PATIENTS
T. Hadjistilianou, S. De Francesco, P. Galluzzi, A. Cerase, A. Renieri, F.
Mari, L. Micheli, M. De Luca, G.Coriolani, C. Menicacci, M. Borri
9 RBp131
OPHTHALMOSCOPIC DIFFERENTIATION OF COATS’
DISEASE FROM RETINOBLASTOMA
Jerry A Shields, Carol L Shields
2149 RBp132
PROTON IRRADIATION FOR RETINOBLASTOMA
W. Sauerwein, B. Timmerman, N. Bornfeld, J. Herault, J. Farr, B.
Zimmermann, A. Wittig
52 RBp133
MULTIPLE PLAQUE TREATMENT IN RETINOBLASTO-
MA
Manoj Parulekar, Randhir Chavan, John Ainsworth, Helen Jenkinson, Dan
Ford, David Spooner, Geoff Heyes
2301 RBp134
ASSESSMENT OF POST-OPERATIVE VOMITING IN
RETINOBLASTOMA PATIENTS AND THEIR SIBLINGS
UNDERGOING EYE EXAMS UNDER ANESTHESIA
Pascal Owusu-Agyemang, Elizabeth Rebllo, Radha Arunkumar, Joseph
Ruiz, Dan Gombos

120 RBp135
MANAGEMENT OF AN ECTOPIC SELLAR TRILATERAL
RETINOBLASTOMA
Rana’a Al-Jamal, Sanna Seitsonen, Ulla Pihkala, Matti Tenhunen, Päivi
Lindahl, Leena Koskinen, Tero Kivelä
350 RBp136
MOLECULAR PATHWAYS OF RETINOBLASTOMA RE-
VEALED THROUGH GLOBAL PROTEOMICS ANALYSES
OF Y79 AND CHLA215 CELL LINES: A DRUG RESIS-
TANCE CASE STUDY
Susan Lee, Robert Fanter, Narine Harutyunyan, Joanne Lee, A Linn
Murphree
1950 RBp137
EVALUATING THE GLYCOLYTIC PATHWAY IN RETINO-
BLASTOMA: A MECHANISTIC APPROACH USING THE
GLYCOLYTIC INHIBITOR 2-DEOXY-D-GLUCOSE IN VIT-
RO AND IN VIVO
Christina L. Decatur, Yolanda Piña, Samuel Houston, Elizabeth Sullivan,
RETINOBLASTOMA
Program
34
Huaping Liu, Theodore Lampidis, Timothy G. Murray
101 RBp138
REGIONAL AND TEMPORAL VARIATIONS IN GENE
EXPRESSION AND VASCULATURE DURING RETINO-
BLASTOMA TUMORIGENESIS AND ITS IMPACT ON
OCULAR TREATMENT
Yolanda Piña, Timothy G. Murray, Christina L. Decatur, Nikesh Shah,
Ludimila Cavalcante, and Samuel Houston

152 RB1
GUIDELINES FOR IMAGING OF RETINOBLASTOMA:
DIAGNOSTIC IMAGING STRATEGY AND
STANDARDIZED MR IMAGING PROTOCOL
P. de Graaf1 , S. Göricke2 , F. Rodjan1, P. Galluzzi3, P. Maeder4 , J.A.
Castelijns1, H.J. Brisse5 on behalf of the European Retinoblastoma
Imaging Collaboration (ERIC) (p.degraaf@vumc.nl)
1. Department of Radiology, VU University Medical Center, Amsterdam,
The Netherlands
2. Department of Diagnostic and Interventional Radiology and
Neuroradiology, University Hospital, Essen, Germany
3. Unit of Diagnostic and Therapeutic Neuroradiology, Azienda
Ospedaliera e Universitaria Senese, Policlinico “Le Scotte”, Siena, Italy
4. Service de Radiodiagnostic et Radiologie Interventionelle, CHUV,
Lausanne, Switzerland
5. Département d’Imagerie, Institut Curie, Paris, France
Purpose. Diagnosis of retinoblastoma is usually established by
the ophthalmologist on the basis of fundoscopy and ultrasound
(US). Together with US, high-resolution MR imaging has emerged
as an important imaging modality for the pretreatment assessment
of retinoblastoma, i.e., for diagnostic confirmation, detection of
local tumor extent and depiction of associated brain abnormalities
(developmental malformation, or trilateral retinoblastoma, i.e.,
intracranial primitive neuroectodermal tumor). MR imaging is
currently performed in the work-up for retinoblastoma in many
institutions around the world. However, diagnostic accuracy for
detection of high-risk features, especially optic nerve infiltration, is
highly dependend on technical quality of MR images. Thus, there is
the need for a standardized protocol with minimal requirements for
imaging retinoblastoma.
Methods. In this imaging guideline, the minimum requirements for
pretreatment diagnostic evaluation of retinoblastoma or mimicking
lesions are presented, according to the consensus reached between
members of the European Retinoblastoma Imaging Collaboration
(ERIC).
Results. The most appropriate techniques for state-of -the-art
diagnostic imaging of a child with leukocoria are reviewed. CT is
no longer recommended. A standardized MR imaging protocol was
developed, including detailed technical requirements and specific
imaging stategies for unilateral and bilateral disease.
Conclusions. Implementation of a standardized MR imaging protocol
for retinoblastoma in clinical practice will allow a state-of-the-art
radiologic evaluation for the detection of tumor extent.
Financial disclosure. None
1831 RB2
CHEMOTHERAPY FOCAL THERAPY AND BEYOND:
THE IMPORTANCE OF CLINICAL TRIALS & THE
ROLE OF A RETINOBLASTOMA CLINICAL STUDIES
CONGLOMERATE
Helen Dimaras1, Ashwin C. Mallipatna2, Brenda L. Gallie1, Helen S.L.
Chan1.(helen.dimaras@utoronto.ca)
1. The Hospital for Sick Children and The University of Toronto
2. Narayana Nethralaya Hospital
RETINOBLASTOMA
Abstracts
35
Purpose
The National Retinoblastoma Strategy Canadian Guidelines for Care
point out the absence of Class A evidence (randomized clinical trials) to
guide therapy. Consequently, consensus recommendations and current
practice at Retinoblastoma Centres underpin the Guidelines. Because
retinoblastoma is rare, few clinical trials have been completed.
Methods. We undertook search of the NIH Clinical Trials Database
using the search term ‘retinoblastoma’ (http://clinicaltrials.gov/
ct2/results?term=retinoblastoma). The results of this search were
categorized purpose, country, and number of centres involved, and
publications that ensued. Results were used to inform suggested
directives for retinoblastoma clinical research.
Results. Our search yielded 54 hits, in which 19 trials studied the
efficacy of a treatment specifically targeting retinoblastoma patients.
Only 6 were multicentre trials, with most participating centres in highincome
countries. There was little representation of middle-income
countries (3/19 studies), and none of low-income countries (0/19
studies), where most children with retinoblastoma reside. Most results
were unpublished; those published were limited in scope and study
numbers.
Conclusions. Clinical trials are the gold standard for evidence-based
care, as they ascertain utility, efficacy and safety of new methods.
Despite this, the few retinoblastoma clinical trials target only a small
proportion of affected children in rich countries, resulting in small
and often homogenous study populations, with results that may
not be applicable to children around the world. As shown in other
pediatric cancers, conducting rigorous, significant, multicentre trials
led by multidisciplinary teams will most effectively improve care for all
retinoblastoma children. We propose a retinoblastoma clinical studies
conglomerate to develop, conduct and evaluate novel clinical studies
that contribute to evidence based care. Since only 9000 children
each year are newly diagnosed, clinical trials could involve all eligible
children.
Financial disclosure. None
1846 RB3
RETINOBLASTOMA: ANALYSIS OF MORTALITY IN
UZBEKISTAN
Z. Islamov, F. Islom (dr_islamov@yahoo.com)
National Center of Oncology, Uzbekistan
Mercer University, United States
Purpose. We sought to analyze mortality from retinoblastoma and
identify factors that predispose to these deaths.
Methods. From 2001 to 2010, 314 patients age

After treatment, 10 patients lived

egression. 2. Advanced tumours show acute ocular signs like resolved
retinal detachment associated with spontaneous regression.
The features that are useful in identifying regression become more
subtle in younger children. Regression may be difficult to detect below
14 months of age, as insufficient time has passed for clues to develop.
As there is a risk of spontaneous reactivation in regressed tumours,
careful monitoring is essential in all such cases.
Financial disclosure. None
47 RB7
RETINOBLASTOMA PATIENTS WITH CHROMOSOME
13Q DELETIONS HAVE INCREASED CHEMOTHERAPY-
RELATED TOXICITIES
R.C. Brennan1, 5, I. Qaddoumi1,5, C. Billups2, C. Odom1, T. Douglas3, W.
Furman1,5 M.W. Wilson4,6 (rachel.brennan@stjude.org)
Department of 1. Oncology, 2. Biostatistics, 3. Surgery and 4. Pathology,
St. Jude Children’s Research Hospital, Memphis, TN, USA. Department
of 5. Pediatrics and 6. Ophthalmology, University of Tennessee Health
Science Center, College of Medicine, Memphis, TN, USA
Purpose. Five-10% of retinoblastoma (RB) patients with RB1 germline mutations
harbor various sized deletions of chromosome 13 (13q-). We reviewed our
experience on a single protocol (RET5) to determine if the 13q- genotype
influences toxicity for these patients during chemotherapy.
Methods. From 2005-2010, 107 RB patients were treated on RET5: stratum
A [low stage; Vincristine(V)/Caroboplatin(C)], stratum B [bilateral; subtenon
C with either VC/Topotecan or VC/Etoposide(E)], and stratum C (unilateral/
primary enucleation; high risk histology received V/Cyclophosphamide(Cy)/
Doxorubicin(D) or VCyD/VCE) with focal therapy as needed. Twelve/107 patients
had 13q- abnormalities (11%). One to two controls matched for age, stratum and
treatment (n=16) were identified for each of 9/12 patients. Grade 3/4 hematologic
(hemoglobin, platelets, and absolute neutrophil count [ANC]), infectious (febrile
neutropenia, bacteremia), and gastrointestinal (GI) toxicities (anorexia/emesis)
were reviewed.
Results. In patients with 13q-, slow ANC recovery delayed chemotherapy (8/9)
more often than in matched controls (9/16), leading to chemotherapy reductions
in 4/9 patients compared with 1/16 controls. GI toxicity was more common
(4/9 vs. 1/16), leading to cessation of chemotherapy in one 13q- patient. For
13q- patients, those with microdeletions (n=5) had significantly more grade 3/4
toxicities (mean 18.4 events) compared with patients who had macrodeletions
(n=5, mean 9 events) (p=0.024). All patients are alive with no evidence of disease
at last follow-up (range 4 to 70 months).
Conclusions. Patients with retinoblastoma and 13q- abnormalities have
more severe chemotherapy-induced toxicities compared to age and therapy
matched controls. These patients may require more intensive supportive care
during chemotherapy, prophylactic cytokine support and dose-reductions in
chemotherapy.
Financial disclosure. None
358 RB8
TOPOTECAN IS EFFECTIVE IN ADVANCED INTRAOCU-
LAR RETINOBLASTOMA WITH MANAGEABLE TOXIC-
ITY
Ibrahim Qaddoumi 1,8, Catherine Billups 2, Clinton F. Stewart 3, Jianrong
RETINOBLASTOMA
Abstracts
37
Wu 2, Katherine Helton 4, Mary McCarville 4, Thomas E. Merchant 4,
Rachel Brennan 1, Barrett G. Haik 5,7, Carlos Rodriguez-Galindo 1,8,
Matthew W. Wilson 5,6,7. (ibrahim.qaddoumi@stjude.org)
Departments of 1. Oncology; 2. Biostatistics; 3. Pharmaceutical Sciences;
4. Radiological Sciences; 5. Surgery; 6. Pathology St. Jude Children’s
Research Hospital, Memphis, TN
Departments of 7. Ophthalmology (Hamilton Eye Institute) and 8.
Pediatrics, University of Tennessee Health Sciences Center, Memphis,
TN
Dr. Rodriguez-Galindo is currently at Dana-Farber Cancer Institute,
Boston, MA
Purpose. To evaluate response rate of topotecan in intraocular
retinoblastoma (RB).
Methods. Patients with bilateral RB in whom at least one eye was
Reese-Ellsworth IV or V were eligible to receive window therapy
consisted that of 2 courses of vincristine and topotecan (VT with G-CSF
support). Patients with ≥ partial response received 3 more courses of VT
alternating with 6 courses of vincristine and carboplatin.
The topotecan dose started at 3 mg/m2/day, and was adjusted to
attain a target systemic exposure of 140 ± 20 ng/ml*hr. Carboplatin
was administered to achieve an area under the curve of 6.5 mg/ml/min.
The dose of vincristine dose was 0.05 mg/kg if age at diagnosis < 12
months and 1.5mg/m2 if age at diagnosis > 12 months.
Results. A total of 27 patients were enrolled with a median age of 8.1
months (range, 0.7 to 22.1 months). Twenty-four of 27 patients responded
(88.9%; 95% Blyth-Still-Casella CI, 71.3%-96.9%). Hematologic toxicity
in the form of grade 4 neutropenia (n=27), grade 3 anemia (n=19)
and grade 3-4 thrombocytopenia (n=16) were observed during the VT
window. Thirteen patients developed grade 3 non-hematologic toxicity.
G-CSF support was added after treating 10 patients. Patients treated with
GCSF (n=17) had a significant shorter duration of grade 4 neutropenia
(median, 7 days) compared to patients without G-CSF (n=10) (median,
24 days) (Wilcoxon rank sum test p

Methods. Six cycles of carboplatin 560 mg/m2 on day 1, etoposide
150 mg/m2 on days 1 and 2, and vincristine 1.5 mg/m2 on day 1 were
prescribed. Patients less than 36 months of age were dosed on a per
kilogram basis (conversion: 30 kg = 1 m2). Toxicity was graded according
to the National Cancer Institute Common Toxicity and Adverse Event
Criteria, Version 3.0.
Results. Five hundred and twenty-one cycles were administered to 93
patients. The median age was 28 months (range, 2-77). Three patients
were under 6 months of age at initiation of chemotherapy. Grade 3-4
myelosuppression (anemia or thrombocytopenia or neutropenia)
was noted in 3% of cycles. Fever and neutropenia occurred in 2 % of
cycles. All other Grade 3 and higher toxicities were seen in less than 1%
of cycles administered. No patient had documented hearing loss and
no patient developed a second malignancy. There were no treatment
related deaths.
Conclusions. Standard dose CEV is associated with a very low rate of
toxicity. Pediatric ophthalmologists and oncologists should not hesitate
to recommend this life preserving treatment.
Financial disclosure. None
1615 RB10
CONSERVATIVE TREATMENT OF INTRAOCULAR
RETINOBLASTOMA: A PROSPECTIVE PHASE II RAN-
DOMIZED TRIAL OF NEOADJUVANT CHEMOTHERAPY
FOLLOWED BY LOCAL TREATMENT
Livia Lumbroso-Le Rouic1, Isabelle Aerts2, David Hajaje3, Christine Lévy-
Gabriel1, Alexia Savignoni3, Nathalie Algret3, François Doz2, Laurence
Desjardins1 (livia.lumbroso@curie.net)
1. Department of ocular oncology, Institut Curie, Paris France
2. Department of oncologic pediatry, Institut Curie, Paris France
3. Department of Biostatistics, Institut Curie, Paris France
Purpose. Intraocular retinoblastoma treatments often associate chemotherapy
and focal treatments. The protocols vary and may combine two or three drugs,
and different number of cycles associated to the local adjuvant treatments. A
first prospective protocol of conservative treatments in our institution showed
the efficacy of the use of two courses of chemoreduction with etoposide and
carboplatin, followed by chemothermotherapy using carboplatin as a single
agent. In order to decrease the possible long term toxicity of chemotherapy
due to etoposide a prospective randomized chemoreduction protocol was
conducted using vincristine and carboplatin vs. etoposide carboplatin
Methods. The study was proposed when intial tumor characteristics
did not allow front-line local treatments. The phase II randomized study
of reduction chemotherapy used treatments vincristin carboplatin
or etoposide carboplatin, and followed by local treatment including
chemothermotherapy with carboplatin only. Primary endpoint was the
need for secondary enucleation or EBRT not exceeding 40% at two years.
Results. 55 children, 65 eyes were included in the study (May 2004- August 2009).
32 eyes (27 children) were treated conservatively in the arm etoposidecarboplatin
and 33 (28 children) eyes in the arm vincristin carboplatin.
At two years after treatment 23/33 (69.7%) eyes were treated and salvaged
without EBRT or enucleation in the arm vincristin-carboplatin and 26/32
(81.2%) in the arm etoposide and carboplatin.
Conclusions. Neoadjuvant chemotherapy by two cycles of vincristine and
carboplatin followed by chemothermotherapy (laser diode hyperthermia
combined to carboplatine as single drug) does not seem to offer an optimal
local control.
Financial disclosure. None
RETINOBLASTOMA
Abstracts
38
24 RB11
OUTCOMES OF 105 EYES WITH INTRAOCULAR RETIN-
OBLASTOMA TREATED WITH SYSTEMIC CHEMO-
THERAPY AND/OR LOCAL THERAPY AS FIRST TREAT-
MENT
Luiz F. Teixeira1,2, Carla R. Donato Macedo2, Virginia L. L Torres1,2,
Camila Hiromi Hashimoto2, Juliana dos Santos Soares2, Clelia M.
Erwenne1,2 (luizfteixeira@hotmail.com)
1. Department of ophthalmology/ Unifesp- Federal University of Sao
Paulo
2. Pediatric Oncology Institute/GRAACC/Unifesp
Purpose. To evaluate the outcome of 105 eyes with intraocular
retinoblastoma treated with conservative treatment.
Methods. Retrospective review of 85 consecutive patients with
intraocular retinoblastoma (34 unilateral cases and 51 bilateral cases).
Results. A total of 105 eyes, 9 group A eyes (9%), 22 group B eyes (21%),
17 group C eyes (16%), 43 group D eyes (41%) and 14 group E eyes (13%)
were treated.
Systemic chemotherapy associated with local therapy was performed
as first treatment in 97 eyes (92%). Periocular carboplatin injection
(20mg/2ml) was used in 13 group D eyes (30%) and in 7 group E eyes
(50%) associated with systemic chemotherapy.
Disease progression was observed in 57% (n=60 eyes) during the first
treatment period (B-32%, C-35%, D-86% and E-86%). Enucleation was
performed in 28 eyes (27%).
Recurrence appeared in 18 eyes from 77 eyes initially saved, after
a mean follow up of 7 months (range 4-12 months). Seven eyes were
enucleated.
Considering all forms of conservative treatments and excluding EBRT,
the final eye salvage rate was respectively: 100%/100% for group A,
95%/95% for group B, 88%/82% for group C, 49%/40% for group D,
54%/54% for group D with periocular carboplatin and 28%/14% for
group E.
A total of 70 eyes (67%) were saved.
Conclusions. Conservative treatment for intraocular retinoblastoma
using systemic chemotherapy associated with local therapy is very
efficient for initial disease. Most of the enucleations (80%) were
indicated during the first treatment period. Recurrence appear most in
the first year of follow up.
Financial disclosure. None
1546 RB12
BRACHYTHERAPY IN THE TREATMENT OF RETINO-
BLASTOMA. EXPERIENCE IN A SPANISH POPULA-
TION
Mónica Asencio-Duran, José Abelairas, José-María Peralta, José-Maria
Fernández-Guardiola, Ernesto Sánchez-jacob (masedur@hotmail.com)
Hopsital La Paz, Madrid, Spain
Purpose. To study the efficacy of brachytherapy in retinoblastoma as a
consolidation treatment in structural and functional terms.
Methods. Descriptive, retrospective and longitudinal study in a series of
86 treatments in 80 retinoblastomas belonging to 67 eyes in a Spanish
reference Hospital.
Results. There were 23 men/34 women, with a medium age at diagnosis

of 10.4 months. 53 % were unilateral, with involvement of the RE in 51
%, and the most frequent symptom was leucocoria (49 %), followed by
strabismus (35 %). The most frequent Reese-Ellsworth groups were Va
(17 cases), IIa (15 cases) and IIb (12 cases). All patients were treated with
VEC, with a 23 % reduction in tumour volume.
76 % of the eyes received only 1 treatment, 21 % 2, and 1,5 % received
3 and 4 treatments. 93 % were carried out once in a tumor, whereas in
6 it was necessary to repeat the treatment. The most used plaque was
Ruthenium in 95 %. The dose was 4000 cGy to the apex in all cases.
With the combined regimen of QR and brachytherapy we have obtained
a survival rate of 100 %, and a 0% rate of metastasis, trilateral
retinoblastoma and second neoplasia with an average follow-up of 2.6 years.
The conservation rate of the globe was high in the group treated with
brachytherapy (66 % opposite to 34 % of non-treated). 95,5 % of the
treated eyes had good tumour response, whereas only 3 were enucleated
due to bad response to the brachytherapy (4,5 %).
The complications associated were the appearance of retinopathy in 61
%, optical neuropathy in 7,6 % and cataracts in 6 % of the cases.
Conclusions. A consolidation treatment is necessary in spite of a good
initial response to chemotherapy. Brachytherapy has demonstrated to
be a “curative” treatment in a wide spectrum of disease due to the great
availability of plaques and isotopes. In addition, the treatments are easy
to realize, short and repeatable, being able to have a certain preventive
effect of enucleation, since it supports more eyes and during more time
than chemotherapy alone. Nevertheless, it is not capable of preventing
the appearance of new tumors and massive seeding, main reasons for
enucleation, and the complication rate is not negligible, being the main
cause of vision loss.
Financial disclosure. None
2130 RB13
THE RADIOTHERAPY IN COMPLEX TREATMENT OF
ADVANCED RETINOBLASTOMA IN CHILDREN
Tatiana Ushakova1, Igor Glekov2, Olga Gopovtzova3, Igor Dolgopolov4,
Alentina Pavlovskaya5, Irina Matveeva6, Vladimir Polyakov3, Geordge
Mentkevich2 (ushtat07@mail.ru)
1. Institute of Pediatric Oncology and Haematology N.N. Blokhin Cancer
Research Center Rams Moscow, Tumors Head and Neck; 2. Institute of
Pediatric Oncology and Haematology, Radiology; 3. Institute of Pediatric
Oncology and Haematology, Tumors Head and Neck; 4. Institute of
Pediatric Oncology and Haematology, BMT; 5. N.N. Blokhin Cancer
Research Center Rams, Pathology Anatomy; 6. N.N. Blokhin Cancer
Research Center Rams, Cytology, Moscow, Russian federation
Purpose. To determine the place of external beam radiotherapy (EBR) in
treatment of children with advanced retinoblastoma (Rb).
Methods. From 2001 to 2008, 65 of patients with Rb were treated in
risk-adapted protocol at our institute. For group I there were 43 patients
with unilateral Rb had undergone primary enucleation. 17 of 43 patients
had insufficiency or minimum tumor invasion choroid and prelaminar
invasion of the optic nerve (Ia – group). The adjuvant therapy was not
administrated for patients of this group. 18 of 43 patients had invasion
into the anterior chamber massive tumor invasion choroid, intra- and
retrolaminar invasion of the optic nerve (Ib-group). The adjuvant CT
included 4 courses of combination of CY, VP-16, Carbo. In addition to
the above, only 4 from 11 patients who had retrolaminar invasion of the
optic nerve received orbital EBR at a dose of 50 Grey. 8 of 43 had tumor
invasion of optic nerve transection and extrascleral extention (Ic-group)
and 7 of 8 received postenucleation EBR (50 Grey), 4 courses of the same
RETINOBLASTOMA
Abstracts
39
CT and high-dose CT included BU and MELPH followed by autologous
peripheral blood stem cells transplantation (APBSCT). For group II there
were 8 patients (unilateral Rb n=6) had initial extraocular disease and
5 of them were treated with neoadjuvant CT and after they underwent
operation followed by CT, EBR and high-dose CT also followed by
APBSCT. Last III group there were 21 patients had undergone organsaving
treatment of advanced intraocular Rb and 10 of them received
combined CT (CY, VP-16, carbo) and EBR at a dose of 46 Gy (IIIa-group).
Results. The patients from group Ia had disease free survival (DFS) of
full 100% with a median period of follow-up of 45 m. The patients from
group Ib had DFS of 89% with a median period of follow-up of 78 m. The
patients from groups Ic and II had DFS of 64% with a median period of follow-up of
56 mos. 4 of 16 patients in groups Ic and II were excluded from study. The patients
from group IIIa had DFS of 80% with a median period of follow-up of 48 m.
Conclusions. Revision of the indications to after initial enucleation in
depends of histopathologic findings was done. EBR is one of effective
methods for preventive of relapses in extraocular Rb after CT and
operation. Combined CT and EBR generally employed in organ-saving
treatment of advanced intraocular Rb.
Financial disclosure. None
1909 Rb14
RESULTS OF PATIENTS WITH UNILATERAL RETINO-
BLASTOMA TREATED WITH INITIAL ENUCLEATION: A
SINGLE INSTITUTION IN BRAZIL
Carla R. Donato Macedo1, Luiz F. Teixeira1,2, Camila H. Hashimoro1,
Virginia L. L Torres 1,2, Juliana dos Santos Soares, Maria T. Seixas1,3,
Maria C. Martins2, Clelia M. Erwenne1,2 (carladonatomacedo@uol.com.br)
1. Pediatric Oncology Institute/GRAACC/Unifesp
2. Ophthalmology Department/Unifesp
3. Pathology Department /Unifesp
Purpose. Evaluation of patients with unilateral retinoblastoma treated
with initial enucleation.
Methods. A retrospective analysis of patients with unilateral
retinoblastoma that underwent enucleation as initial therapy from
2001 to 2011 in a single institution in Brazil. Enucleation was indicated
as primary treatment for patients with massive unilateral disease. The
presence of pathology high risk features, such as massive choroidal
invasion, any degree of concomitant choroid and optic nerve involvement,
tumor involving the optic nerve posterior to the lamina cribosa, was
indication of six cycles of adjuvant chemotherapy. If the surgical margin
was compromised orbit radiotherapy was indicated.
Results. In our data 123 patients had unilateral retinoblastoma (67,2 %
of retinoblastoma patients). Of these, 80 patients underwent enucleation
as initial therapy. Enucleation as single therapy was performed in 43
patients, 37 patients received systemic chemotherapy as adjuvant
treatment and one patient was treated with external beam radiotherapy
with 45Gy. Systemic chemotherapy with carboplatin and vincristine was
given to 25 patients and carboplatin and etoposide to 12 patients.
All the patients are alive with a median follow up of 79 months , with no
recurrence or death.
Conclusions. For patients with unilateral massive disease, enucleation
remains the standard of care. Adjuvant chemotherapy is beneficial for selected
group of patients with higher risk of extraocular dissemination. Ideally, therapy
should be structured to achieve maximal response with minimal toxicity. In our
data, there were no difference in the treatment with carboplatin and vincristine
or etoposide, but further prospective studies must be done.
Financial disclosure. None

1940 RB15
PROTON THERAPY FOR RECURRENT LOCALLY
ADVANCED RETINOBLASTOMA: COMBINED RESULTS
FROM THE RETINOBLASTOMA CENTER OF HOUSTON
& INDIANA UNIVERSITY
D.S. Gombos MD FACS; A.L. Chang MD; D.L. Andolino MD; H.P. Fontanilla
MD; C. Herzog MD; M. Chintagumpala MD; P. Zage MD; R. Hurwitz MD;
P. Chevez-Barrios MD; A. Mahajan MD (dgombos@mdanderson.org)
The Retinoblastoma Center of Houston
MD Anderson Cancer Center
Indiana University
Texas Children’’s Cancer Center
The Methodist Hospital Research Insitute
Baylor College of Medicine
Purpose. Despite significant advances with systemic and local
chemotherapeutic modalities, relapsed retinoblastoma can be salvaged
with adjuvant radiotherapy. We present a cohort of patients treated with
fractionated proton beam radiotherapy for recurrent locally advanced
retinoblastoma.
Methods. A review of all patients with recurrent retinoblastoma treated
with proton beam radiotherapy at MD Anderson Cancer Center and the
Midwest Proton Radiotherapy Institute.
Results. 14 patients / 16 eyes were treated. Median treatment dose was
45 CGE with a median dose to the bony orbit of 24 CGE. Local control
was achieved in 6 of 16 eyes (38%). Median time to local failure was
eight months. Toxicity included peri-orbital erythema, cataract, retinal
vasculopathy and neovascular glaucoma.
Conclusions. Proton beam radiotherapy can be used in the salvage
setting for patients with advanced disease refractory to other treatments
with results comparable to other series.
Dosimetric planning is superior to other modalities.
Financial disclosure. Dr Gombos is a member of the Children’s Oncology Group and has received
reimbursement for travel. He has provided consultation for IMS Health.
1828 RB16
COMPARISON OF HIGH-RISK HISTOPATHOLOGY
BETWEEN UNTREATED AND TREATED EYES OF PA-
TIENTS WITH RETINOBLASTOMA
M.W. Wilson1,2,5, R. Brennan3,6, C. Rodriguez-Galindo3,6, C. Billups4,
B.G. Haik5, I. Qaddoumi3,6 (mwilson5@uthsc.edu)
Departments of 1. Surgery, 2. Pathology, 3. Oncology, and 4. Biostatics,
St Jude Children’s Research Hospital, Memphis, TN, USA
Departments of 5. Ophthalmology and 6. Pediatrics, University of
Tennessee Health Science Center, Memphis, TN, USA
Purpose. To compare high risk histology between untreated and treated
eyes of patients with retinoblastoma.
Methods. A retrospective study identified 177 eyes of 172 patients
enucleated between February 1986 and September 2010. Review of
ocular histopathology focused on high risk features: tumor invasion of
the anterior chamber, iris, ciliary body, choroid (massive), retro-laminar
optic nerve, or sclera, and/or extraocular disease.
Results. 116 eyes of 115 patients were primarily enucleated. The
untreated group had a higher proportion of Reese-Ellsworth (RE) Group
V eyes, 94% versus 59% (p0.19). Forty of the 52 patients were further
treated with adjuvant chemotherapy (n=40) and/or external beam
radiation (n=12). 3 patients died; 2 untreated from metastatic disease
despite adjuvant therapy and 1 with metastatic disease at diagnosis
from complications related to bone marrow transplant.
Conclusions. Despite more favorable Reese-Ellsworth Grouping, treated
eyes with retinoblastoma had an equal risk of harbouring HRH compared
to untreated eyes, committing patients to further adjuvant therapy.
Financial disclosure. None
2 Rb17
PATHOLOGY ASSESSMENT IN UNILATERAL RETINO-
BLASTOMA WITH & WITHOUT HISTOPATHOLOGIC
HIGH-RISK FEATURES & THE ROLE OF ADJUVANT
CHEMOTHERAPY: A COG STUDY
P. Chévez-Barrios1-3,11, R. Eagle1,4, D. Albert1,5, G. Vemuganti6, S.
Krishnakumar7, B. Langholz1, S. Honavar6, J. O’Brien1,8,9, A. Leahey1,8,
K. Matthay1,10, A. Meadows1,8, M. Chintagumpala1,3,11
(pchevez-barrios@tmhs.org)
1. Children’s Oncology Group
2. The Methodist Hospital Research Institute, Houston, TX, USA
3. Retinoblastoma Center of Houston, TX, USA
4. Will’s Eye Institute, Philadelphia, PA, USA
5. University of Wisconsin Hospital and Clinics, Madison, WI, USA
6. LV Prasad Eye Institute, India
7. Vision and Medical Research Foundation, Sankara Nethralaya, India
8. Children’s Hospital of Philadelphia , PA , USA
9. Scheie Eye Institute, Philadelphia, PA, USA
10. University of California San Francisco Medical Center-Parnassus, CA, USA
11. Baylor College of Medicine, Houston, TX, USA
Purpose. Children’’s Oncology Group (COG) finalized a prospective
multicenter international study in patients with unilateral retinoblastoma
undergoing enucleation to determine the prevalence of specific, strictly
defined histopathologic (high-risk) features (HRFs) and the role of
chemotherapy to prevent recurrences.
Methods. Eyes enucleated for unilateral retinoblastoma were submitted
for central review and three pathologists (PCB, RE, DA) independently
reviewed the slides. Additional slides were requested if material was
thought inadequate for interpretation. Central pathology consensus
stratified patients with one or more of the following: posterior uveal
invasion >3mm, any degree of concomitant optic nerve and choroid
involvement, and post-lamina optic nerve involvement. These patients
received chemotherapy; others were observed.
Results. Of 312 patients with central review, 49 patients had their risk
classification changed (13% of initial non-risk had HRFs, 24% of initial
HRFs had non-HRFs). The most important reason for discrepancy was
initial inadequate sections and sampling. 92 patients (29.48%) had HRFs
(post-laminar 16.35%, massive choroidal invasion 13.46 %, concomitant
optic nerve and choroidal invasion 10.58%, others 10.89%) requiring
adjuvant chemotherapy. Three of 312 patients developed recurrences.
Conclusions. The study highlights the importance of histopathology

assessment of HRFs and the adequate handling and sampling of the
enucleated eyes with retinoblastoma. The percentage of patients with
HRFs is higher than reported in developed countries but lower than in
developing countries. This percentage most likely represents a worldwide
average, as this is a multicenter international prospective study.
Financial disclosure. None
2123 RB18
LESSONS LEARNED AFTER 5 YEARS (AND 500 INFU-
SIONS) OF CHEMOSURGERY: PREDICTORS OF SUC-
CESS AND FAILURE
David H. Abramson, MD, Brian P. Marr, MD, Scott E. Brodie, MD, PhD, Ira
J. Dunkel, MD, Sotiria Palioura, MD, PhD, Pierre Gobin, MD (abramsod@
mskcc.org)
Memorial Sloan-Kettering Cancer Center; New York Presbyterian
Hospital; Mt. Sinai School of Medicine, New York, N.Y., USA
Purpose. To identify clinical factors that predict success and failure with
chemosurgery for intraocular retinoblastoma
Methods. Retrospective, single Institution review of 500 technically
successful chemosurgery infusions for retinoblastoma performed
between May 2006 and May 2011.
Results. Ocular survival was 100% for Reese-Ellsworth Groups I, II , III
and IV eyes, 78% for Group V and 100% for ICRB B and C eyes, 76% for D
eyes and 92% for E eyes. Naïve eyes did better than previously treated
eyes (87% vs. 77%) and retinal detachment eyes better than attached
retina (86% vs. 79%). Kaplan Meier ocular survival at 2 years was 83% for
naïve (95%CI=69-96%) and 68.1% for previously treated and resistant
eyes (95%CI=53-83%) (p

4. Fluoroscopy for cannula placement was needed in all cases. Radiation
exposure was far below toxic levels in all cases.
5. Complications mainly involved retinal vascular pruning, often
subclinical and only detected on fluorescein angiography. Occlusion of
ophthalmic/retinal artery (5%) or choroidal vessels (10%) was noted.
Conclusions. IAC provides satisfactory control for less advanced
eyes with retinoblastoma. Retinal detachment often shows complete
resolution. Complications of vascular occlusion should be monitored.
Financial disclosure. None
1527 RB21
COMPARATIVE PHARMACOKINETICS OF TOPOTECAN
AND MELPHALAN AFTER INTRA-ARTERIAL ADMINIS-
TRATIONS IN THE SWINE MODEL
Paula Schaiquevich, PhD, Emiliano Buitrago, Bsc, Ana Torbidoni PhD,
Alejandro Ceciliano, MD, Adriana Fandino, MD, Javier Opezzo, PhD,
Marcelo Asprea, DVM, Sergio Sierre, MD, Flavio Requejo, David H.
Abramson, MD, Guillermo F. Bramuglia, PhD, Guillermo L. Chantada,
MD. (gchantada@yahoo.com)
Hospital JP Garrahan
Consejo Nacional de Investigaciones Cientificas y Tecnicas (CONICET)
Facultad de Farmacia y Bioquimica, Universidad de Buenos Aires,Agencia
de Promocion Cientifica y Tecnologica, Argentina
Memorial Sloan Kettering Cancer Center, New York, NY, USA
Purpose. To compare the vitreous and plasma pharmacokinetics of
topotecan and melphalan after ophthalmic artery infusion (OAI) following
super-selective artery catheterization.
Methods. The ophthalmic artery of 8 Landrace pigs was super-selectively
catheterized and 1 mg of topotecan in one group and 7 mg of melphalan in
the other group were infused over 30 minutes. Serial vitreous specimens
were obtained by microdialysis with simultaneous plasma samples. Drug
levels were measured by HPLC and pharmacokinetic analysis was carried
out with the obtained data. IC50 for each drug were estimated in vitro from
retinoblastoma cell line Y79.
Results. Maximum median vitreous (Cmax) topotecan concentration in
the vitreous was 288 nM, (range 247-303) and that of melphalan 650 nM,
(range 160-1360). The ratio between the vitreous and plasma Cmax and area
under the curve were 15.4 versus 3.4 and 29 versus 3.2 for topotecan and
melphalan respectively. Systemic exposure was low for both drugs (median
plasma Cmax: 0.018 and 0.2 uM for topotecan and melphalan respectively).
The IC50 for topotecan was 10 nM and for melphalan 1000 nM. Topotecan
levels higher than the IC50 were detected for up to 4 hours.
Conclusions. Super-selective OAI resulted in vitreous concentrations of
topotecan that exceeds the IC50, while melphalan levels are slightly below
that range. Topotecan vitreous/plasma concentration is almost 10 times
higher than of melphalan.
Financial disclosure. Universidad de Buenos Aires, CONICET, Agencia de Promocion Cientifica y
Tecnologica, (Argentina). Fund for Ophthalmic Knowledge, New York, USA. Fundacion Natali
Flexer, Buenos Aires, Argentina, Hospital JP Garrahan, Buenos Aires, Argentina
539 RB22
RELAPSES FOLLOWING INTRA-ARTERIAL CHEMO-
THERAPY WITH MELPHALAN
T. Hadjistilianou1, S. De Francesco1, S. Bracco2, P. Galluzzi2, P. Toti3,
P. Gennari2, A. D’Ambrosio4, M. Caini4, D. Galimberti4, A. Cerase2, C.
Venturi2 (hadjistilian@unisi.it)
RETINOBLASTOMA
Abstracts
42
1. Ophthalmology Unit, Retinoblastoma Referral Center
2. Neuroradiology Unit
3. Dept.of Pathology
4. Dept.of Pediatrics
AOUS-Azienda Ospedaliera Universitaria Senese.
Purpose. To report the incidence of relapses following intra-arterial
chemotherapy with melphalan for advanced retinoblastoma at diagnosis
and after systemic neoadjuvant chemotherapy.
Methods. From may 2008 to may 2011, 41 patients (45 eyes, 25 eyes
with relapses and 20 at first diagnosis) have been treated at the Referral
Center for Retinoblastoma (University of Siena) with intra-arterial
chemotherapy using melphalan alone. All patients had an advanced
stage of disease (VB Reese Classification, D ABC Classification). Twenty
eyes were at first diagnosis and twenty five eyes were relapses after
systemic neoadjuvant chemotherapy. All patients had received 3 to 6
infusions of Melphalan. The dose varied from 3 to 7 mg. Five out of 41
had “tandem” therapy for bilateral relapses. All patients received focal
therapy (argon laser, thermotherapy, cryotherapy, and plaques).
Results. Six out of 20 (30%) eyes at first diagnosis had relapses
following intra-arterial chemotherapy with melphalan. Six out of 20 (30%)
underwent enucleation for progressive disease. Six out of 20 (30%)
obtained complete remission; two were lost to follow up. Fifteen out of
25 eyes (60%) which relapsed after systemic neoadjuvant chemotherapy
had new relapses following intra-arterial chemotherapy with melphalan;
one out of 15 eyes (6%) has been successfully treated with focal therapy
alone, fourteen out 15 required one more cycle (3 infusions) of melphalan
and focal therapy. Nine out of 25 eyes (36%) obtained complete remission
with one cycle of melphalan; 1 patient was lost to follow-up.
Conclusions. Intra-arterial chemotherapy with melphalan may represent
a potential treatment option for advanced retinoblastoma, but more
investigations with new chemotherapeutic agents are necessary when
diffuse vitreous and/or subretinal seeding are present.
Financial disclosure. None
2257 RB23
FACTORS INFLUENCING RESPONSE RATE FOLLOWING
INTRA-OPHTHALMIC ARTERY MELPHALAN SALVAGE
THERAPY FOR RELAPSE AFTER PRIMARY TREATMENT
OF RETINOBLASTOMA
John Hungerford1, Judith Kingston2,3, Stefan Brew4, Ashwin Reddy2,
Mandeep Sagoo1,2, Fergus Robertson4, Jane Herod5 (john.hungerford@
btopenworld.com)
1. Oncology Service, Moorfields Eye Hospital
2. Retinoblastoma Service, St Bartholomew’s and the London Hospital
3. Paediatric Oncology Service, Great Ormond Street Hospital
4. Interventional Neuroradiology Service, Great Ormond Street Hospital
5. Anaesthetics Service, Great Ormond Street Hospital, London, UK
Purpose. To evaluate intra-ophthalmic artery Melphalan (IAM) as a
salvage treatment.
Methods. Consecutive, retrospective chart review.
Results. Between December 2008 and May 2011, 39 treatment episodes,
each comprising 1-3 infusions of intra-ophthalmic artery Melphalan
(IAM), were undertaken in 36 eyes of 34 patients for relapsed (34) or
refractory (5) retinoblastoma. Of 34 treatment episodes for relapse,
7 were for vitreous relapse alone whilst a further 10 were for patients
with vitreous disease associated with recurrence of the primary tumour.

Seventeen episodes were for isolated relapse of the primary tumour.
The number of courses of IAM treatment received per episode were 1 (in
3), 2 (in 28) and 3 (in 8) episodes. The dose of IAM varied between 3mg-
7.5 mg depending on age.
With a median follow-up of 16 months post IAM, 10/36 (28%) treated
eyes have required enucleation.
All 5 eyes treated for tumours refractive to primary treatment have
also failed IAM and subsequently required enucleation whilst 4/5 of
the other eyes requiring enucleation had been treated with IAM for an
early relapse within 4 months of previous treatment. The remaining
eye treated after a relapse at 8/12, had no viable tumour at the time of
enucleation.
Of the total of 17 eyes (47%) with vitreous disease 4/17 (23%) have been
enucleated.
Five children had abnormal vascular anatomy, 3 with middle meningeal
artery arising from ophthalmic artery, 2 of whom were enucleated.
Conclusions. Refractory disease or early relapse appear to be the major
factors predicting poor response to IAM applied as a salvage treatment.
Financial disclosure. None
60 RB24
INTRAVITREAL CHEMOTHERAPY WITH MELPHALAN
FOR VITREOUS DISEASE IN ADVANCED RETINOBLAS-
TOMA: EVIDENCE FOR SAFETY AND EFFICACY
Francis L. Munier1, Marie-Claire Gaillard1, Aubin Balmer1, Susan
Houghton1, Maja Beck-Popovic1
1. Jules-Gonin Eye Hospital, Lausanne Switzerland
2. Pediatric Hemato-Oncology Unit, CHUV, Lausanne, Switzerland
Purpose. The presence of active vitreous seeding following state-ofthe-art
conservative treatment of intraocular retinoblastoma leads
in the vast majority of cases to either external beam irradiation (EBR)
and/or enucleation. Intravitreal chemotherapy (IVC) is theoretically
the best route for delivering the highest vitreous concentration of a
chemotherapeutic drug, over intravenous, periocular and ophthalmic
artery chemotherapy. It has however remained highly controversial due
to the risk of tumor exteriorization. In 1995 Kaneko and Susuki were the
first to inject Melphalan in the vitreous of 41 eyes and reported a 51.3%
eye preservation rate.
Methods. We retrospectively reviewed all consecutive patients treated
with IVC between April 2009 and July 2011. IVC was performed on
a weekly basis up to 8 injections of Melphalan /event, using a novel
injection technique characterized by the prevention of vitreous reflux
and sterilization of the needle track. Results. 23 eyes from 23
patients with resistant vitreous seeding, even following ophthalmic
artery Melphalan chemotherapy (13 eyes), were included. Mean followup
was 17 months. Complete seeding regression was achieved in 21
patients. Success rate, defined as absence of enucleation and/or EBR,
was 87% (20/23). No ocular IVC-related complications or extraocular
dissemination were experienced.
Discussion. These preliminary results appear promising in terms of
safety and efficacy with unprecedented success rates for resistant
vitreous disease. It must be emphasized that such a delicate procedure
should imperatively be carried out only in tertiary referral centres, due
to the risk of dissemination. IVC needs to be further investigated and its
indications more precisely defined.
Financial disclosure. None
RETINOBLASTOMA
Abstracts
43
7 RB25
SELECTIVE INTRA-ARTERIAL CHEMOTHERAPY AS A
TREATMENT FOR INTRAOCULAR RETINOBLASTOMA:
ALTERNATIVES TO DIRECT OPHTHALMIC ARTERY
CANNULATION
Michael A. Klufas, MD1, Y. Pierre Gobin, MD1, Brian Marr, MD2, Scott
E. Brodie, MD, PhD2, Ira J. Dunkel, MD2, David H. Abramson, MD2
(mak2049@nyp.org)
1. New York-Presbyterian Hospital/Weill Cornell Medical College, New York,
NY, USA
2. Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Purpose. Report results of intra-arterial chemotherapy for retinoblastoma
when delivery of drug was not via direct cannulation of the ophthalmic
artery.
Methods. Retrospective review of 110 eyes treated with 351 intraarterial
infusions at a single institution identified 18 eyes of 14 patients
who received a total of 67 intra-arterial infusions with at least one
intra-arterial chemotherapy infusion not through the ophthalmic artery
(OA). Alternatives included cannulation of the middle meningeal artery
(MMA), or temporary balloon occlusion of the internal carotid artery
distal to the origin of the OA.
Results. Patient survival: 100%; all patients are alive. Ocular survival:
100%; no eye has been enucleated. Mean follow-up was 12.1 months
(median 11, range 3-28). Eyes received a mean of 3.7 intra-arterial
infusions (median 3, range 2-9). Reese-Ellsworth Group included: RE II,
3 eyes; RE III, 4 eyes; and RE V, 11 eyes (ICRB: Group B, 5; Group C,
3; Group D, 10). Treatment routes included: MMA only, 3 eyes; MMA +
OA, 4 eyes; MMA + balloon, 2 eyes; balloon only, 1 eye; balloon + OA, 7
eyes; balloon + OA + MMA, 1 eye. Intra-arterial chemotherapies included
melphalan, topotecan, and carboplatin. Electroretinogram readings
were: stable, 10 eyes; improved, 3 eyes; reduced, 5 eyes. Neutropenia
(CTCAE v3.0 ≥ grade 3) occurred after 37.5% of treatments. One patient
was hospitalized twice, and once was transfused blood products.
Conclusions. Treatment via the MMA or balloon-assisted infusion allows
salvage of eyes and life, without unacceptable local or systemic side
effects in cases where direct OA cannulation is precluded.
Financial disclosure. None
1448 RB26
INTRA-ARTERIAL CHEMOTHERAPY USING SIMUL-
TANEOUS MULTI AGENT CHEMOTHERAPY, THREE
DRUGS, FOR RESCUE OF EYES WITH INTRAOCULAR
RETINOBLASTOMA
Brian P. Marr, M.D.1 , Y. Pierre Gobin, M.D.2, Scott E. Brodie, M.D. ,
Ph.D.1, Ira J. Dunkel, M.D. 3, David H. Abramson, M.D.1 (marrb@mskcc.org)
1. Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer
Center
2. Division of Interventional Neuroradiology, Department of Radiology,
Neurosurgery, and Neurology, Weill Cornell Medical College
3. Dept. of Pediatrics, Memorial Sloan-Kettering Cancer Center, New
York, N.Y.
Purpose. To report our experience with super selective intraarterial
chemotherapy (SSIAC) using simultaneous multi agent chemotherapy
(three drugs) for advanced intraocular retinoblastoma.

Methods. A retrospective chart review of 26 eyes of 25 patients receiving
three-drug (melphalan, topotecan, and carboplatin) multi agent SSIAC
was conducted between May 2006 and June 2011.
Results. Twenty-six eyes, Reese-Ellsworth group 5b (22) 5a (1) 4a (2)
and 3a (1), received 61 infusions of 3 drug multiagent chemotherapy
for rescue of eyes with advanced retinoblastoma. The dose range for
melphalan was 2.5 to 7.5 mg, 0.3 to 0.6 mg for topotecan, and 30 to 50
mg for carboplatin. The median number of multi drug infusions was 2
with a maximum of 4 and minimum of 1 averaging 2.3 per eye. Fourteen
of 25(56%) patients presented after failing intravenous chemotherapy
(IVC), 2/25(8%) after failing IVC and external beam radiotherapy and
1/25(4%) after failing IVC and plaque brachytherapy. Twenty-four /26
(92)% of eyes were salvage over a mean follow up period of 14 mo. (1-43
mo.) Electroretinogram (ERG) showed improvement greater than in 25mv
in 4/26 eyes (15%), greater than 25-mv loss in 12/26 eyes(46%), no
change greater than 25mv in 10/26 eyes(39%).
Conclusions. We have successfully used three-drug multiagent SSIAC to
rescue eyes that have failed IVC and/ or single or double agent SSIAC.
A significant portion of eyes avoided enucleation and retained ERG
function. . Further investigation into multiagent SSIAC is required to
determine its role in treatment of advanced retinoblastoma.
Financial disclosure. None
1550 RB27
OCULAR COMPLICATIONS OF DIRECT INTRA-OPH-
THALMIC ARTERY MELPHALAN TREATMENT FOR RE-
FRACTORY RETINOBLASTOMA
M Ashwin Reddy1,2, Wisam J. Muen1, Judith Kingston1,3, John
Hungerford2, Fergus Robertson4, Stefan Brew4, Mandeep Sagoo1,2,
Dorothy Thompson5 (mashwinreddy@hotmail.com)
1. Retinoblastoma Unit, Barts & the London NHS Trust, Royal London
Hospital, Whitechapel Road
2. Moorfields Eye Hospital, City Road
3. Oncology Department, Great Ormond Street Children’s Hospital
4. National Hospital for Neurology and Neurosurgery, Queens Square
5. Ophthalmology Department, Great Ormond Street Children’s Hospital,
London, UK
Purpose. We report on patients receiving Intra-ophthalmic Artery
Melphalan (IAM), which was delivered via direct catheterisation of the
ophthalmic artery in cases of retinoblastoma refractory to systemic
chemotherapy +/- radiation .
Methods. All cases undergoing IAM between May 09 to August 10 were
included and the results of ocular complications including reduced
vision were recorded. Minimum follow-up was 11 months.
Results. 15 eyes of 14 patients were treated. 5 developed IIIrd nerve
palsies which resolved within 6 months. 5 patients with prior radiation
developed more severe ocular complications. 2 patients demonstrated
good ERG function post-treatment but a subtle deterioration of patternonset
VEPs. 1 child had her vision reduce from 20/20 to 20/40 with
treatment. 10 of 15 eyes (66%) showed a good tumour response. 2
patients with vitreous seeding required enucleation despite systemic
chemotherapy and intraarterial melphalan.
Conclusions. Potentially amblyogenic complications may occur when
using direct IAM. Complications are exacerbated with prior radiation.
Families undergoing this experimental treatment need to be counselled
accordingly.
Financial disclosure. None
RETINOBLASTOMA
Abstracts
44
438 RB28
LIMITATIONS OF THE INTERNATIONAL CLASSIFICA-
TION IN PREDICTING SUCCESS OF INTRA-ARTERIAL
CHEMOTHERAPY FOR GROUP D/E INTRAOCULAR
RETINOBLASTOMA
Sotiria Palioura, MD, PhD, Y. Pierre Gobin, MD, Scott E. Brodie, MD, PhD,
Brian P. Marr, MD, Ira J. Dunkel, MD, and David H. Abramson, MD (sotiria.
palioura@gmail.com)
Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center,
New York. Currently, Department of Ophthalmology, Massachusetts Eye
and Ear Infirmary, Boston. Division of Interventional Neuroradiology,
Departments of Radiology, Neurosurgery and Neurology, Weill Cornell
Medical College, New York Presbyterian Hospital, New York; Ophthalmic
Oncology Service, Memorial Sloan-Kettering Cancer Center, New York;
and Department of Ophthalmology, Mount Sinai School of Medicine,
New York; Brian P. Marr: Ophthalmic Oncology Service, Memorial
Sloan-Kettering Cancer Center, New York; Department of Pediatrics,
Memorial Sloan-Kettering Cancer Center, New York; David H. Abramson:
Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center,
New York, NY, USA.
Purpose. To report the success rate of superselective ophthalmic
artery chemotherapy for International Classification group D and E
retinoblastoma eyes and to compare it to reported success rates of
systemic chemoreduction.
Methods. Retrospective review of 41 group D (n=33) and E (n=8) eyes of
40 retinoblastoma patients who were treated (May 2006-June 2011) with
intra-arterial chemotherapy as primary treatment. Kaplan Meier data
analysis on ocular event-free (enucleation or external beam radiation)
survival is reported. The PubMed database was searched through June
2011 for studies reporting success rates of systemic chemoreduction for
Group D and E eyes.
Results. The Kaplan-Meier estimates of ocular event-free survival at 2
years were 75.5% (95% confidence interval, 58.2%-92.9%) for group D
eyes and 100% (95% confidence interval, 62.5%-100%) for group E eyes.
The success rates of systemic chemoreduction for group D eyes reported
in the literature range from 23% to 47%. According to our literature
search, all group E eyes were enucleated either at presentation or after
failed systemic chemoreduction and/or external beam radiation.
Conclusions. The high success rate of intra-arterial chemotherapy for
group D and E eyes suggests that the International Classification falls
short when predicting intra-arterial treatment success. It seems that
clinical features used by the International Classification that would deem
an eye “hopeless” for chemoreduction are not predictors of treatment
failure when intra-arterial chemotherapy is used instead.
Financial disclosure. None
2330 RB29
INTRA-ARTERIAL MELPHALAN DOSING REGIMENS
FOR THE TREATMENT OF RETINOBLASTOMA
Timothy G. Murray1, Samuel K. Houston1, Mohammad A. Aziz-Sultan2,
Christina E. Fernandes3, Christina Decatur1, Yolanda Pina1 (TMurray@
med.miami.edu)
1. Bascom Palmer Eye Institute; 2. University of Miami, Department of
Neurosurgery
3. University of Miami, Department of Pediatrics

Purpose. The purpose of this study is to demonstrate dosing protocols for
intraarterial chemotherapy for the treatment of children with retinoblastoma.
Methods. his is an interventional case series of 21 patients with
advanced retinoblastoma (Reese-Ellsworth Vb) treated with intraarterial
chemotherapy and focal laser ablation. Dosing regimens included 3
different strategies. Patients were treated with either: (1) single dose
of 7.5mg if unilateral disease; (2) repetitive treatment with 5mg or
7.5mg for 3 sessions; (3) tandem, bilateral therapy with 7.5mg to the
more advanced eye for a total of 12.5mg. Six patients were treated with
3mg initially, then retreated at higher doses. Patients were evaluated by
indirect ophthalmoscopy and wide-field photography for tumor response.
Results. Patients tolerated treatment well, without severe side effects of
stroke or death. Mild local toxicities were observed, as well 4 cases of
vitreous hemorrhage. However, vitreous hemorrhage occurred in patients
who were treated with lower doses (less than 5mg), then retreated with
higher doses secondary to a lack of response at the lower dose. Systemic
side effects were mild, with mild cytopenias not requiring hospitalization.
75% of patients avoided enucleation or radiation therapy, with patients
treated at lower doses (3 or 5mg) requiring enucleation 36% of the time
versus 0% for those treated at higher doses (7.5mg).
Conclusions. Superselective intraarterial chemotherapy offers an
exciting globe-salvaging technique for the primary and salvage treatment
of retinoblastoma. Standard dosing regimens have not been determined
and vary between institutions. The current study presents dosing
strategies for intraarterial melphalan in the treatment of retinoblastoma
at a single specialized center.
Financial disclosure. None
15 RB30
THE GENERAL HEALTH AND PSYCHOSOCIAL FUNC-
TIONING OF ADULT SURVIVORS OF RETINOBLAS-
TOMA: PRELIMINARY RESULTS OF THE RETINOBLAS-
TOMA SURVIVOR STUDY
Ira J Dunkel1, Jennifer S Ford1, Charles A Sklar1, Kevin C Oeffinger1,
Joanne F Chou1, Yuelin Li1, Danielle Novetsky Friedman1, Mary McCabe1,
Nancy Kline1, Leslie L Robison 2, Ruth A Kleinerman3, Brian P Marr1,
David H Abramson1 (dunkeli@mskcc.org)
1. Memorial Sloan-Kettering Cancer Center, New York, NY, United States,
2. St. Jude Children’s Research Hospital, Memphis, TN, United States, 3.
National Cancer Institute, Bethesda, MD, United States
Purpose. To describe the health status, quality of life, and psychosocial
functioning of adult retinoblastoma survivors.
Methods. A self-report descriptive study (modified CCSS questionnaire
with added VFQ-25 vision questions) was conducted via mail and/or
telephone interview with adult retinoblastoma survivors who had been
treated in New York.
Results. 470 (48%) of 987 potential subjects participated. Mean age at
study entry was 43.4 years (SD 11); 52.1% are female, 90.4% white/non-
Hispanic, and 53.6% had bilateral retinoblastoma. Most were currently
employed full-time (72.2%), married (54.4%), and had at least some
college education (81.0%). The majority endorsed having good overall
health (94.4%), having good eyesight (61.4%), and having no physical
disabilities (61.8%), but 15.4% reported difficulty obtaining health
insurance. A history of psychological dysfunction was reported by 30.7%,
and 35.4% reported having anxiety as a result of their retinoblastoma.
Many worried about passing retinoblastoma to their children (52.3%),
had concerns about their future health (69.9%) or developing a cancer
RETINOBLASTOMA
Abstracts
45
(70.5%), and were dissatisfied with their facial appearance (78.2%).
Participants who reported any disability, poor health, anxiety due to
retinoblastoma, worry about passing retinoblastoma to their children,
concerns about future health or developing a cancer, were significantly
more likely to have been diagnosed with bilateral than unilateral
retinoblastoma.
Conclusions. Most adult retinoblastoma survivors are high functioning
and healthy, but many expressed concerns about the long-term
implications of their retinoblastoma. Future analyses will be conducted
to compare these results to a control (CCSS sibling) population.
Financial disclosure. None
2323 RB31
LATE MEDICAL OUTCOMES IN SURVIVORS OF EXTRA-
OCULAR RETINOBLASTOMA: THE MEMORIAL SLOAN-
KETTERING CANCER CENTER (MSKCC) EXPERIENCE
D. Novetsky-Friedman1, C.A. Sklar1, K.C. Oeffinger1, N.A. Kernan1,
Y. Khakoo1, B.P. Marr2, S.L. Wolden3, D.H. Abramson2, I.J. Dunkel1
(friedmad@mskcc.org)
1. Department of Pediatrics, 2. Ophthalmic Oncology Service; 3.
Department of Radiation Oncology, Memorial Sloan-Kettering Cancer
Center, New York, New York, USA
Purpose. To provide the first report characterizing chronic health
conditions among survivors of extra-ocular retinoblastoma.
Methods. Retrospective analysis of late medical outcomes in 20 survivors
of extra-ocular retinoblastoma diagnosed between 1990 and 2009.
Late effects were graded using the NCI’s CTCAE v4.0 scale. All patients
received intensive multimodality therapy, which included conventional
chemotherapy (n=20, 100%), radiation therapy (n=14, 70%), and/or
high-dose chemotherapy with autologous stem cell transplant (n=18,
90%).
Results. The median follow-up was 10.9 years from initial diagnosis
of intra-ocular RB (range 1.8-18.8 years) and 7 years from diagnosis of
extra-ocular RB (range 0.9-18.4 years). After excluding visual defects,
which were present in 100% of survivors, the most common long-term
complications were hearing loss (n=14, 70%), short stature (n=8,
40%), and neurocognitive delay (n=7, 35%). Eighty-percent of survivors
exhibited ≥2 non-visual long-term effects of any grade. Except short
stature, which was not graded for severity, grade 3-4 toxicities were
limited to: ototoxicity (n=8; n=4 require hearing aids), secondary
malignancies (n=5), ovarian dysfunction (n=1), and unequal limb length
(n=1). Grade 4 secondary malignant neoplasms (SMNs) developed at a
median of 11.6 years after initial diagnosis; two of the five patients died
of their SMN. Cardiac, pulmonary, hepatobiliary or renal toxicities were
not identified in any survivors.
Conclusions. Late effects are commonly seen in survivors of extra-ocular
retinoblastoma but the majority are mild-moderate in their severity.
Longer comprehensive follow-up is needed to fully assess treatmentrelated
chronic health conditions in this population.
Financial disclosure. None
1755 RB32
VARIATION OF SECOND CANCER RISK BY FAMILY
HISTORY OF RETINOBLASTOMA AMONG LONG-TERM
SURVIVORS

Ruth A. Kleinerman1, Chu-ling Yu1, Mark P. Little1, Yi Li2, David H.
Abramson3, Johanna H. Seddon4, and Margaret A. Tucker1 (kleinerr@
mail.nih.gov)
1. Division of Cancer Epidemiology and Genetics, National Cancer Institute,
National Institutes of Health, Department of Health and Human Services,
Rockville, MD
2. Department of Biostatistics and Computational Biology, Dana Farber
Cancer Institute, Boston, MA
3. Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer
Center, New York, NY
4. Ophthalmic Epidemiology and Genetics Service, Tufts-New England
Medical Center, Boston, MA
Purpose. To evaluate the risk of a non-ocular second cancer (SC) in
long-term survivors of retinoblastoma (Rb) according to classification of
germline mutation, based on family history of Rb and laterality.
Methods. We assembled a cohort of 1,852 1-year survivors of
retinoblastoma. SCs were confirmed by pathology reports. Classification
of RB1 germline mutation, inherited or de novo, was inferred by laterality
of Rb and positive family history of Rb. Standardized incidence ratios and
cumulative incidence for all SCs combined and for soft tissue sarcomas,
bone cancers and melanoma were calculated. The influence of host and
therapy related risk factors for SC was assessed by Poisson regression
for bilateral survivors.
Results. We observed a relative risk (RR) of 1.10 (95% Confidence
interval (CI), 0.84-1.44) for SCs in bilateral survivors associated with a
family history of Rb, adjusted for treatment, age and length of followup.
The risk for melanoma was elevated for survivors with a family
history of Rb (RR=2.38, 95%CI 1.04-5.09), but not for bone or soft tissue
sarcomas. The cumulative incidence of SCs at 50 years after diagnosis
of bilateral Rb, with adjustment for competing risk of death, was higher
for survivors with a family history (44%, 95%CI, 35%-54%) than without
(38%, 95%CI, 31-44%).
Conclusions. Rb survivors with bilateral disease and an inherited
germline mutation may be at slightly higher risk of a SC compared
to those with a de novo germline mutation, in particular melanoma,
perhaps due to shared genetic alterations.
Financial disclosure. None
2039 RB33
A NOVEL, ORAL, NON-INVASIVE METHOD OF DELIV-
ERY FOR THE GLYCOLYTIC INHIBITOR 2-DEOXY-D-
GLUCOSE IN THE TREATMENT OF RETINOBLASTOMA
Christina L. Decatur, Yolanda Piña, Samuel Houston, Ludimila Cavalcante,
Theodore Lampidis, Timothy G. Murray (cdecatur@med.miami.edu)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA.
Purpose. The aim of the current study is to assess the impact of oral
delivery of the glycolytic inhibitor 2-deoxy-D-glucose (2-DG) on tumor
burden and hypoxia in the LHBETATAG retinal tumor model.
Methods. The study protocol was approved by the University of Miami
Institutional Animal Care and Use Review Board Committee. LHBETATAG
transgenic mice (n=25) received oral delivery of either 2-DG (0.02%) in
custom made food pellets, or control food pellets without 2-DG, and
were divided into three groups: (1) a group treated for 8 weeks, from 4
to 12 weeks of age (8 weeks early tx); (2) a group treated for 18 weeks,
from 4 to 22 weeks of age (18 weeks early tx); and (3) a group treated
RETINOBLASTOMA
Abstracts
46
for 8 weeks, from 17 to 25 weeks of age (8 weeks late tx). At the time of
enucleation, all eye samples were snap frozen and analyzed for tumor
burden and hypoxia using histopathology and immunohistochemistry
techniques. Percentages of the different variables were statistically
analyzed using ANOVA.
Results. Following oral delivery of 2-DG, there was a significant
difference between all treatment groups on tumor burden (p=0.049). A
78% reduction of tumor burden was found in the 18 weeks early treated
group (p=0.0034). Both the early and late treated groups did not show
any difference from the control (p=0.61 and p=0.58, respectively). A
91% reduction in hypoxia was found in the 18 weeks early treated group
(p=0.061). Although there was a large reduction in hypoxia in both the 8
weeks early and late treated groups (85% and 45%, respectively), they
did not show any difference from the control (p=0.4 and p=0.37, respectively).
Conclusions. Reduction of tumor burden and hypoxia with oral delivery
of 2-DG presents a novel approach as a potential therapeutic treatment
of retinoblastoma. The use of glycolytic inhibitors as adjuvants to
therapeutic strategies, (e.g., chemotherapy, vessel targeting) as a
therapeutic strategy offers a new approach for enhancing current
retinoblastoma treatments.
Financial disclosure. This work was supported by the American Cancer Society and the
University of Miami Sylvester Comprehensive Cancer Center. NIH center grant P30EY014801 and
by the unrestricted grant to the University of Miami from Research to Prevent Blindness.
1352 RB34
VITREOUS INJECTION THERAPY OF MELPHALAN FOR
RETINOBLASTOMA
Shigenobu Suzuki, Akihiro Kaneko (sgsuzuki@ncc.go.jp)
1. Department of Ophthalmic Oncology, National Cancer Center Hospital
2. Yokohama City University Hospital
Purpose. To describe the adverse events and prognosis of retinoblastoma
patients treated with vitreous injection therapy.
Methods. A retrospective review of retinoblastoma patients treated with
vitreous injection therapy until 2009. Injected drug was melphalan, and
the dose ranged from 8 to 24 microgram. All vitreous injections were
performed as salvage treatments, and concomitant treatments were
selected depend on the eye condition: radiotherapy, chemotherapy,
arterial injection, laser therapy, cryotherapy, brachytherapy, and ocular
hyperthermia.
Results. 896 injections were performed for 237 eyes of 227 patients.
Mean follow-up period was 91 months.
Adverse events: Extraocular tumor extension occurred in one eye treated
at the early time of this technique (0.4%), which had anterior chamber
spread and dense vitreous seeds. Systemic metastasis occurred in ten
patients, but nine of them were not related to vitreous injections, and
only in one patient (0.4%) we could not eliminate the association of
vitreous injection. Other adverse events were vitreous hemorrhage (two
eyes, 0.8%), retinal detachment (one eye, 0.4%), chorioretinal atrophy
(two eyes, 0.8%), and iris atrophy (three eyes, 1.3%). There was no case
of endophthalmitis.
Prognosis: In total, 135 eyes (58%) were salvaged. For 83 eyes with active
vitreous seeds without large residual retinal tumors, 68% of eyes were
salvaged. More than half eyes without initial macular tumor kept visual
acuity better than 0.5 (Landolt ring).
Conclusions. Vitreous injection therapy is effective for vitreous seeds,
and causes few adverse events. Good visual acuity means the limited
damage for the eye.
Financial disclosure. None

58 RB 35
A STUDY OF UNILATERAL RETINOBLASTOMA WITH
AND WITHOUT HISTOPATHOLOGIC HIGH-RISK FEA-
TURES AND THE ROLE OF ADJUVANT CHEMOTHERA-
PY: A CHILDREN’S ONCOLOGY STUDY
M. Chintagumpala, R. Eagle, D. Albert, B. Langholz, V.A. Reddy, V.
Khetan, S. Honavar S, J. O’Brien, A. Leahey, K. Matthay, A. Meadows, P.
Chevez-Barrios (mxchinta@txch.org)
Baylor College of Medicine, Houston, Texas; Children’s Hospital of
Philadelphia; University of Wisconsin Hospital; Children’s Oncology
Group;
LV Prasad Eye Institute, Hyderabad, India; Sankaranethralaya, Chennai,
India; University of California San Francisco Medical Center;
The Methodist Hospital, Houston, Texas
Purpose. The COG completed a prospective study in patients with
unilateral retinoblastoma who undergo enucleation to determine the
prevalance of specific, strictly defined histopathologic (high-risk)
features that are predictors of recurrence and the role of chemotherapy
to prevent recurrences.
Methods. All patients who underwent enucleation for unilateral
retinoblastoma were eligible for the study. Pathology slides were
submitted for central review within 21 days of enucleation. Patients with
evidence of one or more high-risk features (posterior uveal invasion
grades IIC and D, concurrent optic nerve and choroid involvement and
post-lamina optic nerve involvement) as determined by central review,
received 6 cycles of chemotherapy consisting of carboplatin, vincristine
and etoposide. All others were observed. All patients were followed for
extraocular or metastatic recurrences.
Results. Patients were enrolled from February of 2005 until May
2010. Of 312 patients with central review, 49 patients had their risk
classification changed. 92 patients with high-risk features received
adjuvant chemotherapy. Three of 312 patients developed recurrences;
two of them had received chemotherapy for massive uveal and postlaminar
involvement, and the third one was observed after enucleation.
Conclusions. Preliminary results of this study suggest that prophylactic
chemotherapy after enucleation for selected patients with unilateral
retinoblastoma can lead to an excellent outcome.
Financial disclosure. None
RETINOBLASTOMA
Abstracts
47

2006 RBp100
RETINOBLASTOMA ASSESSMENT BY DOPPLER
SONOGRAPHY - A FOLLOW - UP STUDY
Maria Teresa B.C. Bonanomi, Osmar C. Saito, Tatiana Tanaka
(mtbonanomi@uol.com.br)
Hospital das Clínicas da Faculdade de Medicina da Universidade de São
Paulo (HCFMUSP) São Paulo - BRAZIL
Purpose. Chistian Andreas Doppler (1803-1853) first described the
physical principle that permits to assess the velocity of an object by
using the change of frequency of waves. This principle was applied in
medicine to study the large vessels in 1957. Color Doppler imaging (CDI)
is an ultrasound technique that combines B-scan images with velocity
information obtained from the Doppler shift of the moving erythrocytes.
The intrinsic circulation of tumors has been reliably imaged in
melanomas before and after treatment. The Purpose of this study is to
assess the retinoblastoma volume and its vascularization by CDI before
and during treatment.
Methods. Retinoblastoma tumors in children with bilateral disease were
studied with the “Toshiba Aplio XG, 16 MHz Transducer. Tumors stage
B or higher were assessed before and after three cycles of the operative
protocol treatment in the HCFMUSP. Tumor volume was calculated
by a multiplication of three manually obtained ultrassonographic
measurements (transversal, longitudinal and antero-posterior in
centimetres) and the mathematical constant 0.52. Vascularization
was indicated as present or absent. Data was compared with the
ophthalmoscopy findings.
Results. There were 14 eligible tumors in 5 children (2 males and 3
female). The mean age of presentation was 10 months (3 to 21months).
Only two lesions were excluded due to unreliable data. The initial mean
volume of the twelve tumors was 0.37cm3 ± 0.68 cm3 (0.004 cm3 to
2.36 cm3). Intrinsic vessels were detected in 9 lesions with mean
volume of 0.49 cm3±0.76 cm3 while in three lesions, mean volume
of 0.01cm3±0.01cm3, tumor vessels could not be demonstrated, the
difference was statistically significant ( p=0.036 Mann-Whitney).The
mean volume at three months was 0.04cm3 ± 0.06cm3 (zero to 0.172
cm3), corresponding to a mean reduction of 86%. The tumor vessels
pattern was exuberant showing variable degrees of turbulence before
treatment. The vascularization at 3 months, considered only in 6 tumors
that were not flat, was still present in 1 (29%) and totally absent in 5.
All 6 tumors showed features of viability at fundus examination in the
third month, meaning opaque tissue sometimes with visible vessels.
Movement of the child and the presence of calcium inside the tumor
hamper the vessel imaging. After treatment, vascularization shrinkage
can be demonstrated by CDI, even in small lesions.
Conclusions. Echodoppler is able to assess size and vascularization of
the tumor in the same session. Intrinsic vessels may be overlooked in
too small tumors. Lack of vascularization in the follow-up does not imply
total involution of the tumor. This diagnostic tool should be considered
in assessing retinoblastoma.
Financial disclosure. None
354 RBp101
PATIENTS WITH UNILATERAL RETINOBLASTOMA AND
HIGH-RISK PATHOLOGIC FEATURES DO NOT REQUIRE
INTENSIVE METASTATIC WORK-UP OR AGGRESSIVE
CHEMOTHERAPY
RETINOBLASTOMA
Posters
48
Ibrahim Qaddoumi1,7, Matthew W. Wilson2,3,6, Catherine Billups4,
Jianrong Wu4, Thomas Merchant5, Barry Shulkin5, Rachel Brennan1,
Barrett G. Haik3,6, Carlos Rodriguez-Galindo1,7, Erin Sullivan1. (ibrahim.
qaddoumi@stjude.org)
Departments of 1. Oncology, 2. Pathology, 3. Surgery, 4. Biostatistics,
5. Radiological Sciences St. Jude Children’s Research Hospital; 6.
Ophthalmology (Hamilton Eye Institute) and 7. Pediatrics, University of
Tennessee Health Sciences Center, Memphis, TN.
Dr. Rodriguez-Galindo is currently at Dana-Farber Cancer Institute,
Boston, MA
Purpose. To describe the work-up, treatment, and outcome of patients
with advanced unilateral intraocular retinoblastoma.
Methods. Patients received up-front enucleation, then were assigned to
Low-Risk (LR), Intermediate-Risk (IR), or High-Risk (HR) groups according
to histology of enucleated eyes. LR patients underwent observation. IR
patients received 4 courses of chemotherapy that included vincristine,
doxorubicin, and cyclophosphamide (VDC). HR patients received 6
courses of chemotherapy: VDC alternated with vincristine, carboplatin,
and etoposide (VCE). The IR and HR groups also received granulocytecolony
stimulating factor.
Results. Fifty patients were treated: LR, 36 patients; IR, 7 patients;
HR, 7 patients. The median age at enrollment was 26 months. All eyes
were classified as Reese-Ellsworth group V. All bone scans (n=79),
lumbar punctures (n=17), and bone marrow aspirates (n=16) were
negative. Chemotherapy-related toxicity was well tolerated. Grades 3
and 4 hematologic toxicities were seen in all patients; grades 3 and 4
nonhematologic toxicities were seen in 7 of 14 patients (in the IR and HR
groups). Chemotherapy dose modifications or delays due to neutropenia
or thrombocytopenia were needed in only 4/67 (6%) courses. Only1
patient in the HR group received radiation therapy.
All patients were alive at the time of analysis; 1 patient was still on active
therapy, and none had experienced recurrence. Median follow-up was
2.5 years (range, 0.2-5.7 years).
Conclusions. Nonmetastatic retinoblastoma with high risk pathologic
features may be cured with a short course of chemotherapy with good
toxicity profile. Thus, intensive metastatic work-up and aggressive
chemotherapy are not required.
Financial disclosure. None
1916 RBp103
TEN YEARS OF EXPERIENCE IN THE TREATMENT OF
INTRA-OCULAR RETINOBLASTOMA IN A SINGLE
INSTITUTION IN BRAZIL
Carla R. Donato Macedo1, Luiz F. Teixeira1,2, Camila H. Hashimoto1, Virginia
L. L Torres1,2, Juliana dos Santos Soares1, Maria T. Seixas1,3, Maria C.
Martin 2, Clelia M. Erwenne1,2 (carladonatomacedo@uol.com.br)
1. Pediatric Oncology Institute/GRAACC/Unifesp
2. Ophthalmology Department/ Unifesp
3. Pathology Department /Unifesp
Purpose. Present clinical and epidemiological features, treatment
modalities and survival of patients with intra-ocular retinoblastoma
Methods. Data as epidemiological features, laterality, international
staging of retinoblastoma, treatment modalities, survival and the
incidence of second malignancies were collected.
The best initial and subsequent treatments were based on whether the
child has unilateral or bilateral disease, the stage of the disease, and
the age of the child.

Results. A total of 183 patients with retinoblastoma, 50,3% male
and 49,7% female. 63% of white ethnicity. Leucocoria was the most
commom initial presentation (65%). 67,2% had unilateral and 33%
bilateral retinoblastoma (243 eyes).
43% of eyes underwent enucleation as primary treatment, of these,
19% received chemotherapy as adjuvant treatment. Chemotherapy
with focal therapy FT was given to 52% of eyes as first treatment,
associated with periocular carboplatin in 8%, brachytherapy in 3%,
external beam radiotherapy (EBRT) in 1%, and one eye received EBRT
and brachytherapy, 12% of these eyes underwent enucleation during
the treatment. 4% received FT alone. None of the patients died with
chemotherapy toxicity.
Intra-ocular recurrent disease occurred in 23% of the eyes with 57% of
eye salvage. 2 patients had recurrent extra-ocular disease with orbital
disease, and bone and bone marrow disease, respectively. Two patients
presented with a bone tumor as second malignancy.
Conclusions. There are several options for treatment of retinoblastoma,
and the multidisciplinary team should be thoroughly familiar with the
indications, technique, and expected Results of all treatment Methods
as well as the expected systemic and visual problems.
Financial disclosure. None
60 RBp104
DIFFERENT CHARACTERISTICS OF GERMLINE AND
NON-GERMLINE RETINOBLASTOMA
Fariba Ghassemi MD1, Hormoz Chams MD1, Siamak Sabour MD2,3,
Reza Karkhaneh MD1, Farzad Farzbod MD1, Mehdi Khodaparast MD1,
Parvaneh Vosough MD4
1. Ocular Oncology and Retina &Vitreous Service, Farabi Hospital,
Tehran University of Medical Sciences.
2. Department of Epidemiology, Faculty of Health, Shahid Beheshti
University of Medical Sciences.
3. Health Promotion and Injury Prevention Research Centre, Shahid
Beheshti University of Medical Sciences.
4. Oncology Service, Mahak Hospital, The Society for Supporting the
Children Suffering from Cancer, Tehran, IR Iran.
Purpose. Retinoblastoma (RB) is a relatively common childhood tumor.
This report discusses clinical characteristics, treatments and outcome
of Germline and Non-germline retinoblastoma patients from a referral
centre in Iran.
Methods. A retrospective study was carried out on cases treated in
Farabi Hospital between 1979 and 2007. The variables analyzed were
age, sex, affected eyes, time of the diagnosis, treatment modalities,
pathological findings and the survival in two groups, i.e. Germline and
Non-germline patients.
Results. We analyzed 557 cases (734 eyes) with a mean age of 32.2
months (Standard Deviation = 22). Male predominance was especially
observed in the Germline group (P< 0.05). There were 380 unilateral
cases (68.6%). Germline cases were different than Non-germline group
in mean age, symptoms, and outcome (P

months. Mean delay between beginning of symptoms and first visit
by a physician was about 3 months. The most common presenting
signs were leukocoria (68%), strabismus (20%) and red eye (7%).
86.8% of unilateral cases and 46.3% of bilateral cases presented
with advanced Rb (groups D-E). Enucleation was done primarily
for 77% of unilateral cases and 35% of bilateral cases. Secondary
enucleation was necessary in 6% and 7% of unilateral and bilateral
cases respectively.
The 5-year cumulative patients survival rate was 94% and 84% for
unilateral and bilateral cases respectively. Overall 5-year survival
rate was 89%.
Conclusions. Prognosis of Rb cases in developing countries is
promising in terms of both globe and patients survival because of
established multidisciplinary coordinated services.
Financial disclosure. None
40 RBp107
EXPERIENCE IN TREATMENT OF METASTATIC &
NON-METASTATIC ORBITAL RETINOBLASTOMA IN A
SINGLE INSTITUTION
Carlos A. Leal, Julieta Robles-Castro, Gabriela Isaac, Vanessa Bosch
(drcarlosaleal@msn.com)
Retinoblastoma Clínic Instituto Nacional de Pediatra Mexico
Purpose. Orbital Retinoblastoma is an infrequent condition in
developed countries, while, on the contrary, in developing countries,
it is diagnosed in 50% of patients. We evaluated the prognosis of
metastatic & no metastatic orbital disease treated with radio- and
chemotherapy.
Methods. A prospective study of the last 15 years regarding treatment
with chemotherapy and radiotherapy.
Results. Five hundred patients with Retinoblastoma were studied.
Thirty percent presented with orbital disease, and of these, 50% were
metastatic. The two-year overall survival of patients with metastatic
disease to the central nervous system was 10%.
The presence of only a positive optic nerve had a better prognosis
than gross orbital disease.
Overall survival of patients with positive optic nerve without
metastatic disease treated with chemo and radiotherapy was 65%.
Neoadjuvancy avoided exenteration and did not affect prognosis.
We evaluated 4 different chemotherapy schemes. The best one was
carboplatin, etoposide, cyclophosfamide.
Conclusions. A positive nerve has a better prognosis than orbital
disease. BMT is necessary for metastatic disease. Carbo vp CFA is
appropiate scheme for advance disease. Neoadjuvancy does not
affect prognosis.
Financial disclosure. None
836 RBp108
ERG MONITORING OF RETINAL FUNCTION DURING
SYSTEMIC CHEMOTHERAPY FOR RETINOBLASTOMA
Scott E. Brodie, M.D., Ph.D.1,4 , Yannis M. Paulus, M.D.1, Mrinali Patel,
M.D.1, Y.
Pierre Gobin, M.D.3, ,1 , Ira J. Dunkel, M.D.2, Brian Marr, M.D.1, David H.
Abramson, M.D.1 (scott.brodie@mssm.edu)
RETINOBLASTOMA
Posters
50
1. Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center,
New York, N.Y.
2. Dept. of Pediatrics, Memorial Sloan-Kettering Cancer Center, New
York, N.Y.
3. Division of Interventional Neuroradiology, Department of Radiology,
Neurosurgery, and Neurology, Weill Cornell Medical College, New York, N.Y.
4. Dept. of Ophthalmology, Mt. Sinai School of Medicine, New York, N.Y.
Purpose. To assess the effect of systemic chemotherapy as initial
treatment for retinoblastoma on retinal function by ERG testing.
Methods. Patients referred for initial treatment of retinoblastoma
who were considered unsuitable for local ablative treatment or intraarterial
chemotherapy received intravenous carboplatin (18.7 mg/kg IV
over one hour) treatment every 3-5 weeks for two to four cycles. ERG
recordings were obtained at baseline and at susbsequent examinations
under anesthesia using a hand-held ganzfeld stimulator, according to
a modified ISCEV standard protocol. The responses to 30-Hz flicker
stimulation were adopted as a proxy for the entire set of ERG responses,
as photopic and scotopic responses were highly correlated. A change in
response amplitude of 25 µV was considered clinically significant.
Results. We report ERG findings in 4 patients (8 eyes) who received
primary IV carboplatin for bilateral retinoblastoma. Retinas were
attached at presentation and throughout IV chemotherapy in all 8 eyes.
All four patients (8 eyes) responded well to initial IV chemotherapy. 30-
Hz flicker ERG responses improved in all eyes, significantly in 6 of 8 eyes
(at least one eye of each patient).
Conclusions. These findings suggest that retinal function may improve
following initial treatment of retinoblastoma with IV carboplatin, quite
apart from improvements due to resolution of retinal detachment. This
observation suggests a deleterious effect on retinal function of the presence
of untreated tumors. The potential for functional improvement argues for
globe-preserving therapy even in the presence of extensive retinoblastoma,
as such eyes may retain significant potential for useful vision.
Financial disclosure. None
2117 RBp109
UTERINE LEIOMYOSARCOMA IN RETINOBLASTOMA:
HOW SHOULD WE COUNSEL OUR PATIENTS?
Jasmine H. Francis1, Ruth A. Kleinerman2, David H. Abramson1
(jasminehfrancis@gmail.com)
1. Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center,
New York, NY;
2. Division of Cancer Epidemiology and Genetics, National Cancer
Institute, National Institutes of Health, Rockville, MD
Purpose.. We have previously reported a substantial excess risk for
leiomyosarcomas (LMS) of the corpus uteri more than 30 years after
diagnosis of hereditary retinoblastoma. Here, we describe updated
patient and risk characteristics for uterine LMS (ULMS) and discuss
patient management relative to these findings.
Methods. A cohort study and retrospective chart review. The excess risk
was calculated as the observed number of uterine leiomyosarcoma minus
the expected number based on general population rates times 10,000.
Results. In our cohort of 900 female retinoblastoma survivors, 8 patients
developed ULMS and seven (87.5%) of these patients had hereditary
retinoblastoma. In this latter group, the average age of ULMS diagnosis
was 41.4 (+/- 7.9 years) and the average age of death was 44.2 (+/-
2.9) years. The excess risk of ULMS was 3.87 per 10,000 women with
hereditary retinoblastoma over all ages. However, as patients enter the

age when at risk for ULMS, this excess risk increased dramatically: to
20/10,000 for female hereditary retinoblastoma patients aged between
30-39 years, and to 27/10,000 for patients aged 40+ years. Cumulative
risk at 50 years of age for ULMS in hereditary retinoblastoma patients
was 3.18% (95%CI 0.91%-7.98%).
Conclusions. There is a substantial excess risk of ULMS in female
hereditary retinoblastoma patients, particularly over 30 years of age.
As more patients survive into their thirties, this number is likely to
increase. Relative to these findings, we provide a perspective on how
best to counsel hereditary retinoblastoma patients on lifestyle risk
factors, early childbearing, screening and even prophylactic measures;
all topics that are important, but not well-established for this group of
patients.
Financial disclosure. None
1700 RBp110
MONITORING AND DISCUSSING QUALITY OF LIFE IN
RETINOBLASTOMA ROUTINE PRACTICE
A.C. Moll, M.I. Bosscha, G. LaRiviere, W.A. Kors, I. Verdonck-de Leeuw, J.
van Dijk, J. Huisman (a.moll@vumc.nl)
VU University Medical Center, Department of Ophthalmology,
Amsterdam, The Netherlands
Purpose. To assess feasibility of routine monitoring of quality of life
(QOL) in retinoblastoma patients and their parents to facilitate early
detection of problems and optimize referral to supportive care.
Methods. Just before the yearly follow-up visit retinoblastoma patients
were asked to complete QOL (SDQ, GHQ28) questionnaires and to
answer some specific retinoblastoma questions via a desktop computer
touch-screen, in separate quiet rooms, supervised by a trained staff
member. Outcomes were discussed face-to-face during consultation
with the ophthalmologists. Feasibility of RetinoQuest was evaluated
using study specific questionnaires.
Results. The questionnaire was completed by 36 patients or parents and
by 2 physicians. Retinoquest increased the awareness of patients, parents
and physicians for psychosocial problems and improved communicating
between patient/parent and physician. The 10 adolescents (11-17 year)
were able to fill in the questionnaire independently. The 20 parents of
young children (4-11 year) were all very content about the attention for
psychosocial problems. The 6 adults (21-62 year) ranged from positive
to neutral to negative. The scores of the QOL questionnaires could be
divided in 3 groups: patients with no problems, patients with known
problems and a third group, who had the most benefit because of the
shared awareness of psychosocial problems. They were immediately
referred to our psychologist, a member of the retinoblastoma team, to
set up an individualized supportive care plan.
Conclusions. Touch-screen computer-assisted health-related quality of
life data collection in retinoblastoma patients has yielded positive Results
it is feasible and can be used for clinical and scientific documentation.
Financial disclosure. None
242 RBp111
DOES PARENTAL SOCIOECONOMIC AND EDUCA-
TIONAL STATUS INFLUENCE RETINOBLASTOMA INI-
TIAL PRESENTATION AND ITS SUBSEQUENT MOR-
BIDITIES?
RETINOBLASTOMA
Posters
51
Daniel Colicchio1,2, Carla D. Macedo2, Juliana dos Santos Soares2, Luiz
F. Teixeira1,2 (dcolicchio@gmail.com)
1. Department of Ophthalmology - Federal University of São Paulo -
UNIFESP - EPM
2. Pediatric Oncology Institute - GRACC - UNIFESP
Purpose. To evaluate the relationship between socioeconomic and
parental educational status with retinoblastoma initial grouping
presentation and its morbidities.
Methods. Retrospective analysis of 105 randomly selected patients
presenting with intraocular retinoblastoma.
The following socioeconomic variables were evaluated through selfanswering
questionnaires: parental educational status, family income,
rural or urban living, State of birth, first person to note any symptom,
first sign/symptom noted.
Results. Analyzing the socioeconomic and environmental correlations
with the tumor stage at the initial presentation and the subsequent
morbidities, the following conditions didn’t have any influence in the
earlier diagnosis and better prognosis: rural or urban living, parental
educational status, family income, State of birth, first sign/symptom
noted. The only variable with statistical meaning showed that when
health care professionals were the first person to note any symptom/
sign, the child presented higher rates of non-group D/E stage (43% vs.
9.3%) and less enucleation rates (50.0% vs. 83.7%).
Conclusions. Although there are some theories that low cultural and
financial aspects are related with risk factors for late presentation of
retinoblastoma, the data collected did not show anything that would
contribute to these theories. It also denoted that the patients didn’t
benefit in prevention or early detection despite the money and education
of their parents, probably because retinoblastoma is a rare disease and
even well-educated groups have little information about it.
Eye examination by health care professionals (eg. Pediatricians,
Ophthalmologists) performed during the entire childhood could be
effective in earlier diagnosis and better prognosis of retinoblastoma.
Financial disclosure. None
1534 RBp112
ARE SOCIOECONOMIC STATUS (SES) AND ETHNICITY
RISK FACTORS FOR THE PRESENTATION OF
ADVANCED RB (RB) IN THE UK?
M. Ashwin Reddy1, Rabia Bourkiza1, Archana Kulkarni2, Manoj
Parulekar2, Phillippa Cumberland3, Mandeep Sagoo1, John Hungerford1,
Jugnoo Rahi3 (mashwinreddy@hotmail.com)
1. Barts and the London NHS Trust
2. Birmingham Children Hospital
3. University College London Institute of Child Health
Purpose. To identify features of patients in the UK with advanced RB as
designated by the International Intraocular RB Classification.
Methods. Retrospective review of sporadic cases presenting with early
RB (B&C) and advanced RB (D&E) over 3 years. SES was identified via
zip code and quintile categories of the Index of Multiple Deprivation:
analysed via multinomial regression.
Results. Of 122 patients, the most deprived had an increased likelihood
of having groups D&E compared to B&C. After adjustment for ethnicity
this was marginally significant in group E (Risk Ratio, most deprived
compared to mid-quintile, 3.7 [95% CI 0.8, 16] p = 0.08). Ethnicity was

not associated with severity of RB.
Conclusions. Directing awareness campaigns to low SES groups may be
of benefit in the UK but ethnicity was not a factor.
Financial disclosure. None
352 RBp113
NEUROPSYCHOMOTOR DEVELOPMENT OF EYE
ENUCLEATED CHILDREN WITH RETINOBLASTOMA
Marcela Bagnatori Braga, Juliana dos Santos Soares, Carla R. Donato
Macedo, Walquyria de Almeida Santos (mabagnatori@uol.com.br)
Purpose. Retinoblastoma is the most common malignant ocular tumor
of childhood, usually presenting before four years of age. The incidence
in Brazil shows an average of 3 to 5 cases/million in children younger
than 14 years old.
Currently, the cure rates of Retinoblastoma are higher because
of the advances in diagnosis and treatment. However, aesthetic,
ophthalmologic, neurocognitive and social effects, as well as visual
impairment, makes eye enucleated children with Retinoblastoma
likely to show deficits in Neuropsychomotor Development and/or
Sensory Integration. The Purpose is present the possible changes in
Neuropsychomotor Development and/or Sensory Integration of eye
enucleated children with Retinoblastoma.
Methods. A study was made at the Pediatric Oncology Institute - GRAACC
/ UNIFESP - São Paulo - Brazil. Two assessment tools were used, the
PEDI and the Sensory Profile.
Results. It was possible to check that children in this sample showed
deficits in Neuropsychomotor Development and/or Sensory Integration.
These changes were graduated from minimal to maximum – following
the assessments used.
Conclusions. From this study was contested an alteration in
Neuropsychomotor Development and/or Sensory Integration of these
children related to the disease, treatment and consequences in the
sensorial and motor system of this population.
Financial disclosure. None
345 RBp114
NURSING GUIDELINES FOR SELF-CARE OF PATIENTS
WITH PROSTHETIC RETINOBLASTOMA: AN EXPERI-
ENCE REPORT
Juliana dos Santos Soares, Carla R.D. Macedo, Adriana M. Duarte
(js_soares3@hotmail.com)
Purpose. Retinoblastoma (RB) is a rare cancer in retina, representing
about 4% of all malignancies of childhood. The treatment modalities
include chemotherapy, focal therapy, radiation and enucleation,
according to the stage of disease. Upon receiving news of the
enucleation, child and family tend to be fragile and frightened by the
surgical procedure, the change in self-image and lifestyle after surgery.
In this context, the role of nurse has great value in educating the patient
and family providing support to deal with the new situation. The Purpose
is to report the experience lived by a Nurse at a Brazilian Hospital of
Pediatric Oncology with the orientation of a child with RB and her family
for self-care after enucleation.
Methods. It is an experience report. About 19 queries were held with
the child and her family immediately after enucleation indication and
throughout the adaptation process in the period from September
2010 to July 2011. With appropriate language for the child age and
RETINOBLASTOMA
Posters
52
parents understanding, graphics and ocular prosthesis models were
used for explanation of the whole procedure and required care after
enucleation.
Results. Subjects discussed in the meetings varies since surgery
procedure to prosthetic eye care. After 10 months of starting the treatment,
child and family became more calm and confident when talking about the
experience of enucleation. The child showed to be able to perform selfcare
under supervision of her mother.
Conclusions. The sections were productive, with effective participation
of patient and parents. As a result of these encounters, a plan for
systematic and individualized care was prepared, with quality, by
identifying the real needs of the child submitted to enucleation and her family.
Financial disclosure. None
33 RBp115
EYE SALVAGE RATE IN 166 PATIENTS WITH INTRAOC-
ULAR RETINOBLASTOMA
Luiz F. Teixeira1,2, Carla R. Donato Macedo2, Virginia L. L Torres1,2,
Camila H. Hashimoto2, Rubens Belfort Neto1,2, Juliana dos Santos
Soares2, Clelia M. Erwenne1,2 (luizfteixeira@hotmail.com)
1. Depatment of Ophthalmology, Federal University of Sao Paulo-
UNIFESP
2. Pediatric Oncology Institute/GRAACC/UNIFESP
Purpose. To evaluate the eye salvage rate in 166 patients with
intraocular retinoblastoma.
Methods. Retrospective review of 217 consecutive eyes with intraocular
retinoblastoma (115 unilateral cases and 51 bilateral cases).
Results. A total of 217 eyes, 9 group A eyes (4%), 22 group B eyes
(10%), 17 group C eyes (8%), 53 group D eyes (24%) and 116 group E
eyes (54%) were treated.
112 eyes (52%), (10 eyes (19%) of group D and 102 eyes (88%) of group
E) were treated with enucleation as primary treatment.
Systemic chemotherapy and/or local therapy were performed as first
treatment in 105 eyes (46%), (9 group A, 22 group B, 17 group C, 43
group D and 14 group E). External beam radiotherapy (EBRT) was used
for major tumor recurrence. Analysing conservative therapies the eye
salvage rate considering all forms of treatments and excluding EBRT
was respectively: 100%/100% for group A, 95%/95% for group B,
88%/82% for group C, 49%/40% for group D and 28%/14% for group
E.
From 217 eyes diagnosed with intraocular disease, 70 eyes (32%) were
saved.
The final eye salvage rate was 100% for group A (n=9), 95% for group
B (n=21), 88% for group C (n=15), 40% for group D (n=21) and 3% for
group E (n=4).
Conclusions. Advanced intraocular disease, group D and E represent
more than 70% of the intraocular disease treated in our institution
with a high rate of primary or secondary enucleation. Groups A, B and
C present excellent outcomes but represent only 28% of the eyes.
Financial disclosure. None
933 RBp116
SYSTEMIC CHEMOTHERAPY IN THE MANAGEMENT
OF RETINOBLASTOMA
John D. McKenzie1, Nisha Sachdev1, Sandra Staffieri1,4, Kary Suen1 , James
E. Elder1,2, John Heath2,3, Peter Downie3 (jdmckenzieonline@gmail.com)

1. Department of Ophthalmology, Royal Children’s Hospital, Melbourne
2. Department of Paediatrics, University of Melbourne
3. Children’s Cancer Centre, Royal Children’s Hospital, Melbourne
4. Centre for Eye Research Australia, University of Melbourne
Purpose. To demonstrate the utility of utilising systemic chemotherapy
as adjunctive therapy for the management of retinoblastoma.
Methods. Nine consecutive patients treated with systemic
chemotherapy are described. All have presented with differing
classification of retinoblastoma (according to International
Classification or Retinoblastoma) including 3 with optic nerve
involvement at presentation. No patients had bone marrow infiltration
or CSF involvement.
Results. Each case will be presented individually. Chemotherapy
regimes varied according to “aggressiveness” of tumour and whether
optic nerve invasion was evident. The number of cycles of chemotherapy
changed according to the clinical response as evident on serial EUA’s.
Conclusions. Systemic chemotherapy has revolutionised the
management of retinoblastoma, providing excellent tumour regression
allowing focal therapy to be applied. This therapy frequently avoids
the previously “gold standard” of enucleation.
Financial disclosure. None
2225 RBp117
COMPARISON BETWEEN OPHTHALMIC ARTERIAL
INJECTION THERAPY AND CHEMOREDUCTION AS
PRIMARY THERAPY FOR INTRAOCULAR RETINOBLAS-
TOMA
Takashi Yamane, Nobuyuki Suzuki, Makoto Mohri (joecool777jp@
yahoo.co.jp)
Saiseikai Yokohamashi Tobu Hospital
Purpose. For retinoblastoma patients, we have performed ophthalmic
arterior injection therapy (OPAI) by using melphalan from 1988. We
compared OPAI as primary therapy with OPAI after chemoreduction
retrospectively, and examined the validity of OPAI.
Methods. 128 eyes were treated by chemoreduction (VEC 2-6 course)
before OPAI. ICRB A: 2, B: 45 C: 22 D: 46 E: 13 eyes
49 eyes were treated by OPAI from start of therapy. A: 2, B: 30, C: 5, D:
9, E: 3 eyes
Each eye preservation rate was examined. Most of OPAI were combined
with laser therapy.
Results. Eye preservation rate was as follows:
OPAI after chemoreduction, A: 100%, B: 82% C: 59% D: 41% E: 31%.
OPAI as primary therapy, A: 100%, B: 93% C: 40% D: 78% E: 33%
Conclusions. Ophthalmic arterial injection chemotherapy using
melphalan can expect eye preservation more than an equivalence
compared with chemoreduction following OPAI. We would like to add
statistically examination furthermore.
Financial disclosure. None
942 RBp118
INTRA-ARTERIAL CHEMOTHERAPY FOR RETINOBLAS-
TOMA: FIRST AUSTRALIAN EXPERIENCE
John D. McKenzie1, Nisha Sachdev1, Sandra Staffieri1,4, James E. Elder1,2, John
Heath2,3, Peter Downie3, Peter J Mitchell5,6 (jdmckenzieonline@gmail.com)
RETINOBLASTOMA
Posters
53
1. Department of Ophthalmology, Royal Children’s Hospital; 2. Department
of Paediatrics, University of Melbourne; 3. Children’s Cancer Centre, Royal
Children’s Hospital;
4. Centre for Eye Research Australia, University of Melbourne; 5.
Department of Radiology, Royal Melbourne Hospital, Parkville; 6.
Neurointervention Service, University of Melbourne, Melbourne
Purpose. To present the first Australian case of using intra-arterial
chemotherapy for primary treatment of Retinoblastoma
Methods. A child was referred with leukocoria to Royal Children’s Hospital in
Melbourne. Examination revealed a large lobulated posterior segment mass
occupying more than 50% of the globe. IOP and anterior examination was
normal. MRI showed no extrascleral extension, no optic nerve involvement.
LP, Bone Marrow Aspirate and trephine biopsy was normal.
Standard treatment of primary enucleation was offered to the parents,
which was denied. Globe preservation was strongly desired by the parents
due to the child’s previous history of self-induced wound dehiscence.
It was decided that intra-arterial chemotherapy could be offered as
primary therapy for this large retinoblastoma. Clinical Ethics Committee
approval was obtained to offer this treatment.
Results. Four cycles of intra-arterial chemotherapy (Melphalan 5mg,30
min infusion) were administered monthly. Serial EUA’s and MRI’s were
arranged during this time period. Apart from significant sterile orbital
cellulitis after the inital treatment the intra-arterial chemotherapy was
well tolerated. MRI following the fourth cycle revealed an inferotemporal
orbital mass on the revealed a retinoblastoma deposit. Bone marrow
aspirate and trephine biopsy showed bone marrow infiltration. He
has subsequently had 4 cycles of quadruple systemic chemotherapy
(Vincristine, Etoposide, Carboplatin, Cyclophosphamide).
Conclusions. Intra-arterial chemotherapy for retinoblastoma is an
emerging treatment modality as primary therapy for retinoblastoma;
however, as this case illustrated, further study into the precise
chemotherapeutic agents that should be used and frequency for these
require to be established.
Financial disclosure. None
817 RBp119
OUTCOMES OF GROUP D RETINOBLASTOMA EYES
J.L. Berry1, H. Almarzouki2, S.R. Bababeygy1, T.H. Lee1, 2, R. Jubran2,
A.L. Murphree1, 2 (jesse.berrymd@gmail.com)
1. Doheny Eye Institute, Los Angeles, CA
2. Childrens Hospital of Los Angeles, Los Angeles, CA
Purpose. To determine treatment outcomes of Group D retinoblastoma eyes.
Methods. Retrospective chart review.
Results. All patients diagnosed with retinoblastoma and classified as
Group D in at least one eye during a ten-year period from January 1, 2000
to December 31, 2009 at Childrens Hospital Los Angeles were included.
107 eyes of 94 patients were included in the study; 44 patients had
unilateral disease and 50 had bilateral disease, 13 of which had bilateral
Group D disease. 50 of 107 Group D eyes were enucleated primarily. 57
eyes were treated with systemic and local chemotherapy, as well as local
consolidation. 27 of 57 eyes (47%) were salvaged with chemotherapy and
consolidation therapy. 30 eyes had recurrences; 5 eyes were enucleated
and 25 eyes were treated with 36 Gy intensity modulated radiotherapy
(IMRT). Of the 25 irradiated eyes, 20 (80%) were salvaged. 5 eyes were
enucleated after IMRT treatment. Two patients died from retinoblastoma,
1 from a midline primitive neuroectodermal tumor and the other from CNS
metastases without ocular recurrence. Final visual acuity ranged from
20/20 to light perception with 10 eyes having 20/80 vision or better. 4
eyes had 20/200 vision. Mean length of follow up was 42.6 months.

Conclusions. During a ten-year period, 47 of 57 (82%) treated Group D
eyes were salvaged. 47% (27/57) were salvaged with systemic and local
chemotherapy alone. 20 of 57 (35%) eyes required IMRT for salvage.
In our series, systemic treatment for retinoblastoma even in advanced
eyes can salvage a majority of eyes, many with functional vision.
Financial disclosure. Doheny core grant support: NIH Grant EY03040 and Research to Prevent
Blindness
417 RBp120
THE FIRST EXPERIENCE OF RETINOBLASTOMA TREAT-
MENT WITH THE USE OF SUPERSELECTIVE INTRA-AR-
TERIAL CHEMOTHERAPY IN RUSSIA
S.V. Saakyan1 , S.B. Yakovlev2, G.L. Kobyakov2, N.K. Serova2, A. Jarwa1
(svsaakyan@yandex.ru)
1. Moscow Helmholtz Research Institute of Eye Diseases
2. Moscow Scientific Neurosurgery Institute named after Burdenko
Purpose. Evaluate the effectiveness of SSIAC in treatment of patients
with multifocal retinoblastoma with vitreal seeding.
Methods. 12 children of 1- 6 years of age with retinoblastoma of Stages
D and E, including 9 children with binocular retinoblastomas, were
managed. 10 patients underwent unsuccessful systemic chemotherapy
previously. On average 3 single injections of SSIAC using a 50 mg dose
of carboplatinum were conducted in each case.
Results. Preservation of 8 eyes was achieved with the use of SSIAC (1
eye in a patient with monocular retinoblastoma and 7 eyes in patients
with binocular retinoblastoma), resulting in 67% ocular preservation
rate. Observed findings included a decrease in prominension and
a development of calcifications in the main focus and the vitreal
seedings.
Conclusions. Conclusion: Our first experience demonstrated that SSIAC
allows for ocular preservation and improves the effectiveness of therapy
in children with advanced stages of retinoblastoma.
Financial disclosure. None
2317 RBp121
MISSING INTRAOCULAR BLUSH IN AN ANGIOGRAPHY
OF AN ADVANCED INTRAOCULAR RETINOBLASTOMA
M. Holdt, S. Göricke, E. Biewald, M. Schündeln, N. Bornfeld, M.
Schlamann (markus.holdt@uk-essen.de)
Department of Ophthalmology, Department of Radiology, Department
of Pediatric Oncology
Purpose. To find out therapeutical option for a single case with
advanced bilateral sporadic retinoblastoma (both eyes classified E)
Methods. In a case of a 13 month old girl with bilateral sporadic
retinoblastoma, both eyes were classified E (International Intraocular
Retinoblastoma Classification) or Vb (Reese-Ellsworth-Classification)
without light perception. All examinations including MRI showed no
extraocular pathology. The more affected eye was primary enucleated
without revealing histopathological risk factors. The attempt to
preserve the less affected eye with superselective ophthalmic artery
chemotherapy was discontinued due to not demarcated intraocular
blush beside expanded exophytic tumor masses with retinal
detachment. Angiography showed broad perfusion of periocular
tissues like lacrimal gland or nasal mucosa and a strong anastomosis
RETINOBLASTOMA
Posters
54
to anterior meningeal artery. This eye was also primary enucleated
without revealing histopathological risk factors. Hence no adjuvant
chemotherapy, no other therapy was necessary.
Results. The approach of superselective ophthalmic artery
chemotherapy has limitations when intraocular blush is not
definable.
Conclusions. Primary enucleation with histopathological confirmed
absence of risk factors still offers a therapy without any additional
burden in these rare cases with clinical advice for germline mutation.
Financial disclosure. None
2004 RBp122
CONSERVATIVE TREATMENT OF INTRAOCULAR
RETINOBLASTOMA: A PROSPECTIVE PHASE II TRIAL
FOR BILATERAL RETINOBLASTOMA WITH MACULAR
OR PARAMACULAR INVOLVEMENT
Christine Levy-Gabriel 1, Livia Lumbroso-Le Rouic 1, Isabelle Aerts 2,
David Hajaje3, Alexia Savignoni 3, Nathalie Algret 3, François Doz 2,
Laurence Desjardins 1 (christine.levy@curie.net)
1. Department of ocular oncology, Institut Curie
2. Department of oncologic pediatry, Institut Curie
3. Department of Biostatistics, Institut Curie, Paris France
Purpose. Intraocular retinoblastoma treatments often associate
chemotherapy and focal treatments. The protocols vary and may
combine two or three drugs, and different number of cycles associated
to the ocular treatments. In order to decrease the possible long term
sequels of laser scars for macular or paramacular tumors, a protocol of
laser reduction was initiated.
Methods. Monocentric prospective phase II study including children
with bilateral retinoblastoma and macular or paramacular involvement.
The protocol combines 6 cycles of of 3 drugs (vincristin carboplatin and
etoposide), diode laser thermotherapy sparing macula is associated
from the third cycle.
Results. 19 children, 23 eyes with 23 macular or paramacular tumors were
included in the study (July 2004-Sept 2009). Six tumors were treated
with chemotherapy alone, 2 of them presented a local recurrence. 17
tumors were treated with diode laser hyperthermy sparing macula, one
of them reccured. 20 eyes were salvaged without EBRT, 1 needed EBRT,
and 1 eye was enucleated
Conclusions. Six cycles of of 3 drugs (vincristin carboplatin and
etoposide) associated with diode laser thermotherapy sparing macula
achieves good local control while probably decreasing laser macular
scar. Visual function evolution will need longer follow-up.
Financial disclosure. None
1857 RBp123
EXTRACTION OF RADIATION-INDUCED CATARACT IN
PATIENTS WITH RETINOBLASTOMA
Z. Islamov, F. Islom (dr_islamov@yahoo.com)
National Center of Oncology, Uzbekistan
Mercer University, United States
Purpose. Analysis of Results of radiation-induced cataract extractions in
patients with retinoblastoma.

Methods. From 2001 through 2010, 314 patients age 2 but

30 RBp127
TREATMENT MODULATION IN RETINOBLASTOMA TUM-
ORIGENESIS AND ITS IMPACT ON TUMOR BURDEN
Timothy G. Murray, Samuel Houston, Christina L. Decatur, Nikesh Shah,
Ludimila Cavalcante, and Yolanda Piña (tmurray@med.miami.edu)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA.
Purpose. The Purpose of the current study is to examine vessel targeting,
chemotherapy, and mammalian target of rapamycin (mTOR) inhibitor
agents in LHBETATAG retinal tumors and their impact on tumor burden.
Methods. Group A: Ten-week-old, LHBETATAG mice (n=30) received a
single subconjunctival injection of anecortave acetate (AA; 1200, 600,
300, and 150 µg) delivered to right eyes only. Group B: Ten-week-old,
LHBETATAG mice (n=30) received a single subconjunctival injection of
AA (600, 300, and 150 µg) delivered to right eyes only, either during
a cycle of carboplatin (six subconjunctival deliveries) or after the
completed cycle. Carboplatin was delivered at the subtherapeutic
concentration of 62.5 µg. All animals were euthanatized at 16 weeks of
age, and the eyes were examined histopathologically. Group C: Eighteenweek-old,
LHBETATAG mice received (n=30) subconjunctival injections
of rapamycin once weekly for two consecutive weeks (0.00333, 0.167,
3.33, and 6.67 mg/kg). Tumor sections were analyzed for tumor burden
with immunohistochemistry techniques.
Results. A statistically significant reduction in tumor burden was
detected after a single periocular injection of AA. The reduction of
tumor burden followed a U-shaped dose-response curve. Tumor burden
was significantly decreased when AA and carboplatin were combined.
However, varying doses and delivery schedule of these agents had
significant impact on the effectiveness of the combined treatment.
The most effective scheme was delivering a low dose (150-300 µg) of
AA after a complete cycle of carboplatin. Reduction in tumor burden
were significantly different between rapamycin doses and control
(pA, at the (-23) position of the
intron 9 of the coding sequence of the retinoblastoma gene, RB1. This

ase substitution has not been previously documented as a germ line
mutation associated with retinoblastoma.
Conclusions. Bio-informatic analysis suggests this single base
substitution creates a cryptic splice donor site in intron 9 leading
to aberrant transcription of the RB1 gene. The significance of this
mutation and mechanism to explain the incomplete penetrance is not
known.
Financial disclosure. None
601 RBp130
CNS ABNORMALITIES IN RTB PATIENTS
T. Hadjistilianou1, S. De Francesco1, P. Galluzzi 2, A. Cerase2, A. Renieri3,
F. Mari3, L. Micheli1, M. De Luca1, G.Coriolani4, C. Menicacci1, M. Borri1
(hadjistilian@unisi.it)
1. Ophthalmology Unit, Retinoblastoma Referral Center; 2.
Neuroradiology Unit; 3. Genetics; 4. Dept.of Pediatrics, AOUS-Azienda
Ospedaliera Universaitaria Senese
Purpose. The presence of CNS abnormalities on MR images in a large
group of consecutive patients with retinoblastoma is evaluated. Mental
retardation and congenital brain anomalies are reported in patients with
retinoblastoma, mostly in combination with 13q deletion syndrome.
Pineoblastoma is the most important and “life threatening” condition
associated with hereditary retinoblastoma, but recent studies suggest
an association with pineal cysts.
Methods. MR images of 320 consecutive patients with retinoblastoma
from 2000 to 2010 were evaluated by neuroradiologists for tumors,
structural anomalies, myelinization, and coincidental findings. Clinical
records were reviewed for laterality, heredity, and the presence of the
13q deletion syndrome.
Results. The hereditary group (patients with bilateral and unilateral
proved RB1-germline mutation) included 42 (48%) out of 87 patients.
Nine patients had 13q deletion syndrome. Normal findings on brain
MR images were seen in 307 (96%) patients. One pineoblastoma was
detected in a patient with hereditary retinoblastoma. One arachnoid
cyst in a sporadic unilateral RTB girl. One cerebral and corpus callosum
atrophy, 3 pineal cysts were detected (2 non hereditary, 1 in 13q deletion
syndrome). Corpus callosum agenesis was found in 3 patients (2 13q
deletion syndrome, 1 hereditary RTB), corpus callosum hypoplasia in 3
patients (2 twins, 1 sporadic RTB), both in combination with 13q deletion
syndrome.
Conclusions. Pineoblastoma is associated with hereditary retinoblastoma,
and structural brain abnormalities are associated not only to patients
with the 13q deletion syndrome. Pineal cysts can be detected in patients
with sporadic retinoblastoma and/or with 13q deletion syndrome.
Financial disclosure. None
9 RBp131
OPHTHALMOSCOPIC DIFFERENTIATION OF COATS’
DISEASE FROM RETINOBLASTOMA
Jerry A. Shields, Carol L. Shields (jerryshields@comcast.net)
Wills Eye Institute, Philadelphia PA
Purpose. Coats’disease (CD) and retinoblastoma (RB) are clinically
similar but it is important to differentiate them for clinical and legal
reasons. This study was done to elucidate the clinical differentiate of
these 2 conditions.
RETINOBLASTOMA
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57
Methods. All patients with RB (>1500 cases) and CD (>200 cases)
seen over 30 years were reviewed to establish relative clinical criteria that
serve to differentiate them.
Results. Clinical features that differed in CD and RB included nature of
the pupillary reflex, color of subretinal fluid, and caliber and distribution
of the retinal blood vessels. The pupillary reflex and color of subretinal
material is generally yellow in CD and white to gray with RB. Macular
involvement with CD generally shows yellow lipoproteinaceous
exudation, whereas macular involvement with RB shows a white mass
without exudation. The retinal blood vessels in CD are irregular in
caliber, whereas in RB the retinal vessels are more uniformly dilated and
more tortuous. The blood vessels in CD tend to remain visible from the
posterior pole to the peripheral fundus, whereas the vessels in RB tend
to disappear as dip into the adjacent or underlying neoplasm.
Conclusions. Despite their superficial similarities, CD and RB have
ophthalmoscopic features that differ from one another. Recognition of
these differences can avoid erroneous diagnosis, misdirected therapy,
and legal repercussions.
Financial disclosure. None
2149 RBp132
PROTON IRRADIATION FOR RETINOBLASTOMA
W. Sauerwein1, B. Timmerman2, N. Bornfeld3, J. Herault4, J. Farr2, B.
Zimmermann1, A. Wittig5 (w.sauerwein@uni-due.de)
1. University Duisburg- Essen, University Hospital Essen, Department of
Radiation Oncology, Essen, Germany; 2. WPE gGmbH, Essen, Germany;
3. University Duisburg- Essen, University Hospital Essen, Department of
Ophthalmology, Essen, Germany
4. Cyclotron Biomédicale, Centre Antoine-Lacassagne, 06200 Nice,
France
3. University Duisburg- Essen, University Hospital Essen, Department of
Ophthalmology, Essen, Germany; 5. Klinikum der Philipps-Universität
Marburg. Department of Radiation Oncology Marburg, Germany
Purpose. External beam radiotherapy is a curative treatment in
retinoblastoma leading to excellent functional Results at long term but
burdened by the appearance of secondary cancers. Chemotherapy often
has to be locally supported by coagulative treatments jeopardizing
visual acuity. Furthermore, an increasing number of publication reports
secondary malignancies after cytotoxic drugs in retinoblastoma
patients. There is a need for radiotherapy techniques that reduce the
irradiated volume limiting the dose to the eyeball and sparing normal
tissues. Brachytherapy using radioactive plaques is a well-established
modality but limited to small tumor lesions. Proton therapy offers a
hypothetical approach for further improvements.
Methods. Only 4 retinoblastoma patients received proton treatment by
our group in the last decade. Three were able to collaborate and the
treatment was performed similar to conventional proton irradiation for
uveal melanoma. A five-year-old boy suffered from a recurrent tumor
close to the papilla after a full course of external beam irradiation.
After enucleation of one eye in early childhood, we had to treat a late
recurrence on the other eye that involved not only the retina but also
the anterior chamber. Finally a primary retinoblastoma of the posterior
pole in an adult was irradiated by protons. Another indication was
the irradiation of the orbit in a 1-year-old boy by a ventral field after
enucleation of an eye with involvement of the orbit.
Results. In the first case, vitreal spreading occurred 2 years after PT
and the eye was lost. The histomorphological evaluation showed a
complete control of the proton-irradiated lesion. In the second case,
irradiation with 50 Gy in 25 fractions resulted in a complete remission

(follow-up 5 years). Two years after PT, the adult patient had a
recurrent tumor that was controlled by brachytherapy. Proton therapy
of the orbit in very young patients leads to bad cosmetic Results and
should be avoided.
Conclusions. Further efforts are necessary to find techniques that can
be applied in very young children unable to collaborate. The technique
has to include positioning without using ionizing radiation. In principle,
a dose distribution limited to the eye and not damaging the growing
bones can be achieved using two fields and an isocentric gantry.
Financial disclosure. None
52 RBp133
MULTIPLE PLAQUE TREATMENT IN RETINOBLASTOMA
Manoj Parulekar, Randhir Chavan, John Ainsworth, Helen Jenkinson, Dan
Ford, David Spooner, Geoff Heyes (manojparulekar@aol.com)
Birmingham Children’s Hospital, and University Hospitals Birmingham, UK
Purpose. 106 Ruthenium plaque brachytherapy is a useful tool in salvage
treatment of retinoblastoma with limited lateral irradiation and ease of
use. To determine the safety and efficacy of multiple 106 Ruthenium
plaque treatment for eyes with persistent or recurrent retinoblastoma.
Methods. Retrospective review of all children treated in a single
retinoblastoma centre over an eight year period (2002-2010).
Results. 26 tumours in 21 eyes required brachytherapy as salvage
treatment. 11.5mm (65%), 15.5mm (23%) and notched (12%) plaques
were used. 52% tumours regressed after application of one plaque,
and 25% required a further plaque to control disease. Although some
radiation related complications were noted in the 5 eyes receiving more
than one plaque, 4 of these eyes were salvaged with useful vision (
mean visual acuity 0.6 logMAR).
Conclusions. The main aim of salvaging eyes was achieved in most
cases treated with multiple plaques. Despite the risk of complications,
this high risk strategy might be suitable is some cases to avoid
enucleation.
Financial disclosure. None
2301 RBp134
ASSESSMENT OF POST-OPERATIVE VOMITING IN
RETINOBLASTOMA PATIENTS AND THEIR SIBLINGS
UNDERGOING EYE EXAMS UNDER ANESTHESIA
Pascal Owusu-Agyemang, Elizabeth Rebllo, Radha Arunkumar, Joseph
Ruiz, Dan Gombos (poagyemang@mdanderson.org)
University of Texas M.D. Anderson Cancer Center; Retinoblastoma Center
of Houston
Purpose. To evaluate the incidence of emesis in the PACU in pediatric
patients undergoing eye exams of minimal stimulation under
anesthesia.
Methods. We analyzed data from ophthalmologic procedures that
did not involve a surgical incision, laser, and cryotherapy from our
Automated Anesthesia Information System. Between January 2006 and
July 2010 we found 76 patients with a diagnosis of or need for screening
for retinoblastoma. Our endpoint was administration of an antiemetic
or the documentation of emesis in the PACU. Descriptive statistics were
used in data analysis.
Results. Sixty-three percent of patients received prophylactic antiemetics:
40/76 (53%) received ondansetron alone, 6/76(8 %) received
RETINOBLASTOMA
Posters
58
both ondansetron and dexamethasone, and 2/76 (2.6%) patients
received dexamethasone alone. The overall incidence of emesis in this
study group was 1.3% (1/76 patients). The incidence of emesis in the
group of patients receiving anti-emetics and those not receiving antiemetics
was 0% and 3% respectively.
Conclusions. In our study where identified risk factors were present
but with essentially no surgical stimulation the incidence of POV in the
PACU was lower than the baseline of 9% with no risk factors2. However,
the discomfort associated with POV may justify prophylactic anti-emetic
administration.
Financial disclosure. None
120 RBp135
MANAGEMENT OF AN ECTOPIC SELLAR TRILATERAL
RETINOBLASTOMA
Rana’a Al-Jamal, Sanna Seitsonen, Ulla Pihkala, Matti Tenhunen, Päivi
Lindahl, Leena Koskinen, Tero Kivelä (ranaa.aljamal@hus.fi)
Departments of Ophthalmology, Paediatrics and Oncology, Helsinki
University Central Hospital, Helsinki, Finland
Purpose. To report successful management of an ectopic sellar
trilateral retinoblastoma.
Methods. A 10-month-old girl with strabismus and leukokoria had
bilateral retinoblastoma (Group E, OD; group D, OS). MRI revealed an
enhancing 20x24 mm sellar tumour which protruded to the suprasellar
cistern and pushed the chiasm. Spinal MRI, bone marrow aspirate and
lumbar puncture revealed no metastases.
Results. Combination chemotherapy was started eight days after
diagnosis.
The 1st, 3rd and 5th cycles consisted of intravenous topotecan (2 mg/
m2 on days 1 and 2), cisplatin (120 mg/m2 on day 1), vincristine (2 mg/
m2 on day 3) and intrathecal thiotepa (5 mg on day 5). In the 2nd cycle,
cisplatin was substituted with carboplatin (560 mg/m2 on day 1) and
the 4th cycle consisted of vincristine, topotecan and thiotepa.
The intraocular tumours responded promptly and were consolidated
with TTT. Before the 2nd cycle, she underwent autologous
hematopoietic stem cell harvesting. Stem cell rescue was executed at
5 and 7 months, preceded by high dose chemotherapy (carboplatin
500 mg/m2, thiotepa 300 mg/m2, and topotecan 2 mg/m2 on days
1-3). Stereotactic radiotherapy was given between the stem cell
transplantations (6 MV photons, 1.8 Gy fraction size, 17 fractions) from
a linear accelerator (30.6 Gy) from five conformal dynamic fields.
Latest brain and spinal MRI 3 years after termination of treatment show
full regression. Binocular VA is 20/40. She receives growth hormone,
thyroxine and hydrocortisone substitution.
Conclusions. The patient is one of three known longer term survivors
after an ectopic sellar trilateral retinoblastoma.
Financial disclosure. None
350 RBp136
MOLECULAR PATHWAYS OF RETINOBLASTOMA RE-
VEALED THROUGH GLOBAL PROTEOMICS ANALYSES
OF Y79 AND CHLA215 CELL LINES: A DRUG RESIS-
TANCE CASE STUDY
Susan Lee1,2, Robert Fanter1, Narine Harutyunyan1, Joanne Lee1, A Linn
Murphree1,3 (sulee@chla.usc.edu)

1. Children’s Hospital Los Angeles; 2. Department of Pathology and
Laboratory Medicine;
3. Department of Ophthalmology, Keck School of Medicine, University of
Southern CA, Los Angeles, CA, USA
Purpose. To provide molecular insights into retinoblastoma using global
proteomics analyses of retinoblastoma cell lines, Y79 and CHL215.
Methods. Cell lines were characterized by DIMSCAN drug testing
using carboplatin and topotecan and proteomic analyses using liquid
chromatography coupled to tandem mass spectrometry (LC-MS/MS).
Results. At all concentrations and time points tested, CHLA215 were
much more drug resistant than Y79. One stark example was the
comparison between Y79 and CHLA215 survival fractions, 0.016 versus
0.86, after 4 days in 24 micromolar carboplatin.
The cellular proteomes of Y79 and CHLA215 were characterized using
LC-MS/MS. 724 proteins were identified, with 410 proteins (57 %) in
common. Of these, 27 have been previously shown to have a role in
tumorigenesis. Additionally, 199 and 115 proteins were identified as
unique to Y79 and CHLA215, respectively.
Multiple members of the glutathione-S-transferase (GST) pathway were
shown to be upregulated in CHLA215, including GSTP1, GSTM2, and
GSTM3.
Conclusions. Though retinoblastoma has been associated with cancer
predisposing mutations in the RB1 gene for more than 30 years, there
is still a need to understand the molecular processes of retinoblastoma,
particularly those independent of the RB1 mutation.
Here we have shown that unbiased proteomic analyses can identify
that the GST pathway is upregulated in drug resistant CHLA215
retinoblastoma. This pathway has been implicated in drug resistance of
other malignancies such as breast and lung cancer.
These studies will form the foundation for targeted studies on the
molecular differences between retinoblastoma tumors and their clinical
implications.
Financial disclosure. None
1950 RBp137
EVALUATING THE GLYCOLYTIC PATHWAY IN RETINO-
BLASTOMA: A MECHANISTIC APPROACH USING THE
GLYCOLYTIC INHIBITOR 2-DEOXY-D-GLUCOSE IN VIT-
RO AND IN VIVO
Christina L. Decatur, Yolanda Piña, Samuel Houston, Elizabeth Sullivan,
Huaping Liu, Theodore Lampidis, Timothy G. Murray (cdecatur@med.
miami.edu)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, USA.
Purpose. To assess the molecular mechanism of glycolytic inhibition: (1) in
vivo employing the LHBETATAG retinoblastoma (RB) animal model, and (2)
in vitro using two established human RB cell lines Y-79 and WERI-Rb-1.
Methods. (1) 17-week-old mice received one periocular injection of
2-deoxy-D-glucose (2-DG; 500 mg/kg). Two hours post injection,
animals were euthanized and crude retinal tumor extracts were analyzed
for HIF and HKII expression. (2a) Y-79 and WERI-Rb-1 cells were exposed
to either normoxia or hypoxia (0.5% O2) conditions for 24 hours. Cells
were harvested and basal levels of HIF and HKII were measured. (2b)
To assay 2-DG cytotoxicity at increasing concentrations, Y-79 and WERI-
Rb-1 cells were treated with 3 mM, 6 mM, or 12 mM of 2-DG and placed
in a hypoxic chamber for 72 hours.
RETINOBLASTOMA
Posters
59
Results. (1) There was a 41% reduction in HKII levels and 37% increase
in HIF levels compared to the non-treated tumors (p

8.30-9.00 Poster presentations ECOp101-111
(Moderator: S. Frenkel, H. Tsuji)
9.30-11.00 papers
(Moderators: B. Esmaeli, S. Heegaard)
1621 EC1
EVALUATION OF THE “HEDGEHOG” SIGNAL-
LING PATHWAYS (SHH, PTCH-1, GLI-1 LEVELS) IN
SQUAMOUS AND BASAL CELL CARCINOMAS OF THE
EYELIDS AND CONJUNCTIVA.
Hayyam Kiratli, Ali Rıza Cenk Çelebi, Figen Söylemezoğlu
1246 EC2
MARGIN CONTROL IN EYELID TUMOR EXCISION: IS
FROZEN SECTION OPTIMAL?
David T.L. Liu, Kelvin K.L. Chong, Gary M.K. Tse, N.M. Luk, Dennis S.C. Lam
1407 EC3
RADIOTHERAPY FOR MANAGEMENT OF MEDIAL
CANTHAL BASAL CELL CARCINOMA
Hatem Krema, Caroline Chung, Evelyn Herrmann, Rand Simpson, David
Payne, Normand Laperriere
1756 EC4
DOES THE SIZE OF EYELID SEBACEOUS CARCINOMA
CORRELATE WITH NODAL METASTASIS AND SURVIVAL?
Bita Esmaeli, Qasiem Nasser, Hilda Cruz, Melissa Felman, Carla L.
Warneke, Doina Ivan
2244 EC5
LYMPHOMA OF THE EYELIDS – A NATION-BASED
STUDY
Steffen Heegaard, Peter K. Rasmussen, Elisabeth Ralfkiaer, Lene D. Sjö
and Jan U. Prause
143 EC6
DIAGNOSIS AND FOLLOW-UP OF OCULAR SURFACE
SQUAMOUS NEOPLASIA BASED ON NON-INVASIVE
IN VIVO BIOPSY USING CONFOCAL MICROSCOPY
J. Oscar Croxatto, Carolina Gentile
1459 EC7
OCULAR SURFACE SQUAMOUS NEOPLASIA IN A
PATIENT WITH HIV INFECTION: REGRESSION AFTER
RESTARTING ANTIRETROVIRAL THERAPY WITH
DEVELOPMENT OF SPECIFIC T-CELLS
M. Marinkovic, A. Rengifo Coolman, M.J..P Schoenmaekers-Welters, S.
van der Burg, M.J. Jager, G.P.M. Luyten
Wednesday November 16, 2011
EYELID, CONJUNTIVA & ORBIT
Program
61
12 EC8
RUTHENIUM PLAQUE THERAPY IN THE MANAGEMENT
OF CONJUNCTIVAL SQUAMOUS CELL CARCINOMA
INVADING THE EYE
Priscilla L Ballalai, Virginia L Torres, Roberto Segretto
1059 EC9
INVASIVE SQUAMOUS CELL CARCINOMA OF THE
CONJUNCTIVA TREATED BY PROTONS
W. Sauerwein, J. Herault, P. Chauvel, H. Westekemper, R. Darawsha, B.
Zimmermann, C. Maschi, A. Wittig, L. Brualla, J-P. Caujolle
1505 EC10
PROSPECTIVE STUDY OF SENTINEL LYMPH NODE BI-
OPSY FOR CONJUNCTIVAL MALIGNANT MELANOMA
Victoria M.L. Cohen, Maria Tsimpida, Norbert Avril, John L. Hungerford,
Grahem Moir
2202 EC11
EXENTERATION FOR CONJUNCTIVAL MELANOMA IN
LIVERPOOL: 1993-2010
Nihal Kenawy, Sarah E. Coupland, Bertil E. Damato
2311 EC12
TEENAGE ELEVATED CONJUNCTIVAL LESIONS IN THE
PLICAL AREA – LYMPHOMA OR REACTIVE LYMPHOID
HYPERPLASIA?
Shahar Frenkel, Jacob Pe’er
11.00-11.20 BREAK
11.20-12.45 Papers: Other Intraocular Tumors
(Moderators: J. Elizalde, J. Shields)
1933 OT1
CLINICAL SURVEY OF 3680 SOLID AND CYSTIC TU-
MORS OF THE IRIS
Carlos Bianciotto, Carol L. Shields, Swarupa Kancherla, Mayerling
Suriano, Margaret V. Shields, Priya Sharma, Jinali Patel, Jerry A. Shields
111 OT2
CLINICAL AND OCT FINDINGS ON SYMPTOMATIC
MACULAR NAEVI TREATED WITH INTRAVITREAL ANTI-
VEGF
Sonia A. Callejo, Mikael Sebag, Marc Blouin, Christine Corriveau
1340 OT3
RISK FACTORS FOR GROWTH OF POSTERIOR UVEAL
MELANOCYTIC LESIONS WITH THICKNESS GREATER
THAN 2 MM IN 161 CONSECUTIVE PATIENTS
Patrick De Potter, Audrey Noel, Jacques Jamart

6 OT4
THE EXPANDING SPECTRUM OF RETINAL VASOPRO-
LIFERATIVE TUMOURS
Jerry A. Shields, David Reichstein, Arman Mashayekhi, Carol L. Shields
518 OT5
DIFFUSE CHOROIDAL HEMANGIOMA: CLINICAL
MANIFESTATIONS AND VISION OUTCOME IN 60
PATIENTS
H.K. Li, C.L. Shields, N. Ni, J. Iturralde, J.A. Shields
504 OT6
SD-OCT AND AUTOFLUORESCENCE CHARACTERIS-
TICS OF CANCER-ASSOCIATED AUTOIMMUNE RETIN-
OPATHY
Prithvi Mruthyunjaya and Kathryn L. Pepple
1801 OT7
NEAR-INFRARED AUTOFLUORESCENCE OF
NEUROFIBROMATOSIS TYPE 1
Minoru Furuta, Tetuju Sekiryu, Tomohiro Iida
2340 OT8
VASOPROLIFERATIVE LESIONS (VPLS) OF THE RETINA
AND THE OPTIC DISC
Vicktoria Vishnevskia-Dai, Josef Moisseiev, Amir Elhalel, Iris Moroz,
Roth Sigal, Mordechai Rosner
2113 OT9
VITREORETINAL SURGERY FOR TRACTIONAL
COMPLICATIONS ASSOCIATED WITH JUXTAPAPILLARY
COMBINED HAMARTOMA OF THE RETINA AND
RETINAL PIGMENT EPITHELIUM
Javier Elizalde
206 OT10
RISK OF RADIATION RETINOPATHY IN PATIENTS WITH
ORBITAL AND OCULAR LYMPHOMA
Megha Kaushik, Jose S. Pulido, Khushboo K. Agrawal, Steven E. Schild,
Scott Stafford
2324 OT11
VITREORETINAL LYMPHOMA TREATED BY
INTRAVITREAL INJECTIONS OF METHOTREXATE: 80
EYES OVER 14 YEARS
Jacob Pe’er, Shahar Frenkel
2329 OT12
COMBINATION INTRAVITREAL RITUXIMAB AND
METHOTREXATE FOR PRIMARY VITREORETINAL
LYMPHOMA
David J. Wilson, and Justine Smith
EYELID, CONJUNTIVA & ORBIT
Program
62
12.45-13.15 Poster presentations OTp101-110
(Moderator: J. Pe’er, D. Gombos)
13.15-14.15 LUNCH
14.15-14.35
Keynote Lecture: Dr. Steffen Heegaard
LYMPHOMA OF THE OCULAR ADNEXA.
14.35-17.35 Rapid Fire Cases
(Chair J. Shields)
Retina
(Moderators: C. Shields, A. Schefler)
2128 RF1
RETINOBLASTOMA
David H. Abramson
1941 RF2
EXTRA-OCULAR RETINOBLASTOMA WITH INVASION
OF THE OPTIC CHIASMA: CAN BE SAVED?
Camila H. Hashimoto
54 RF3
SEVEN-YEARS-OLD BOY WITH PERSISTENT PAINFUL
RED RYE AND PARS PLANA LESION
Patricia Chévez-Barrios
1830 RF4
MALIGNANT TRANSFORMATION OF RETINOCYTOMA
Eduardo F. Marback
1937 RF5
SUDDEN VITREOUS HAEMORRHAGE
Tero Kivelä
2044 RF6
SPONTANEOUS ISCHEMIA AND PIGMENT RELEASE
IN AN ADVANCED CASE OF RETINOBLASTOMA
Dan Gombos
121 RF7
SUPRA-SELECTIVE CATHETERIZATION OF THE
OPHTHALMIC ARTERY FOR INTRA-ARTERIAL
CHEMOTHERAPY INJECTION IN RETINOBLASTOMA
Vicktoria Vishnevskia-Dai
1957 RF8
PARANEOPLASTIC VITELLIFORM RETINOPATHY
M. Turell

Uvea
(Moderators: B. Damato, M. Sagoo)
214 RF9
CILIARY BODY TUMOR IN A CHILD
Adriana C. Fandiño
418 RF10
RETINAL DETACHMENT AFTER FNAB
Arturo Irarrazaval
2047 RF11
DIFFUSE MELANOCYTIC NEOPLASM IN A 9 YEAR-OLD
FEMALE WITH OCULODERMAL MELANOCYTOSIS
Amy C. Schefler
2251 RF12
A CASE OF ADENOMA OF THE NON-PIGMENTED
CILIARY EPITHELIUM CAUSING NEOVASCULAR
GLAUCOMA
Vasilios P. Papastefanou
1934 RF13
CHOROIDAL NEVUS...CHOROIDAL NEVUS?
Miguel A. Materin
2312 RF14
MUSHROOM SHAPED TUMOR. MELANOMA OR NOT?
Jerry Shields
104 RF15
AMELANOTIC INTRAOCULAR LESION IN A 26 YEAR-
OLD FEMALE PATIENT
Luiz F. Teixeira
2328 RF16
PIGMENTED ANTERIOR SEGMENT TUMOR IN A
YOUNG MAN
Carolina M. Gentile
2254 RF17
AMELANOTIC CHOROIDAL MASS EXTENDING TO THE
FOVEA
Zelia M Correa
29 RF18
PIGMENTED CHOROIDAL TUMOR
Jacob Pe’er
2028 RF19
RAPID REGRESSION OF POST-BRACHYTHERAPY
CHOROIDAL MELANOMA IN RESPONSE TO INTRAVIT-
REAL AVASTIN
Peter Hovland
EYELID, CONJUNTIVA & ORBIT
Program
63
1845 RF20
LIGHT AND ELECTRON MICROSCOPY OF TWO CILIARY
BODY TUMORS
Lynn Schoenfield
513 RF21
RETINAL DETACHMENT AFTER TTT FOR ACTIVE CHOR-
OIDAL MELANOMA
Prithvi Mruthyunjaya
55 RF22
TREATMENT OF DIFFUSE IRIS MELANOMA
Bertil Damato
62 RF23
LATE RECURRENCE OF A PRESUMED IRIS MELANO-
MA
John McWhae
45 RF24
HOLY TUMOR
Carol Shields
1848 RF25
RESECTION OF AN IRIS TUMOR
Arun D Singh
410 RF26
MELANOMA OR MELANOCYTOMA?
Arturo Irarrazaval
Eyelid
(Moderators: H. Demerci, M. Naseripour)
1655 RF27
MALIGNANT TRANSFORMATION OF CELLULAR BLUE
NEVUS IN AN 8-YEAR-OLD GIRL
Alexander P. Moulin
1618 RF28
ORBITAL MELANOMA AND NAEVUS OF OTA
Victoria M.L. Cohen
558 RF29
MANAGEMENT OF A RARE ADNEXAL TUMOR
Sonul Mehta
Conjunctiva
(Moderators: E. Midena, M. Manquez)

19 RF30
AMELANOTIC CONJUNCTIVAL TUMOR - MALIGNANT OR
BENIGN?
Priscilla Ballalai
1809 RF31
65-YEAR OLD WOMAN WITH DIFFUSE CONJUNCTIVAL
NEOPLASM
J. William Harbour
23 RF32
DIFFUSE CONJUNCTIVAL MALT LYMPHOMA
Shahar Frenkel
Orbit
(Moderators: B. Esmaeli, P. de Potter)
1510 RF33
PAEDIATRIC TUMOUR WITH SCLERAL INVASION
Maria Tsimpida
1227 RF34
STEREOTACTIC-GUIDED TRANSCRANIAL
CRYOEXTRACTION OF CAVERNOUS HEMANGIOMA IN
THE ORBITAL APEX
Mordechai Rosner
1352 RF35
MYSTERIOUS ORBITAL CASE
Patrick De Potter
1802 RF36
ORBITAL INVOLVEMENT FROM AN UNUSUAL
CONDITION
Manquez Maria
112 RF37
REPONSE OF METASTATIC PANCREATIC
ADENOCARCINOMA TO CHEMOTHERAPY
Scott Oliver
127 RF38
A CASE OF TEARING AND SWELLING OF THE EYE
AFTER RESOLUTION OF ORBITAL CELLULITIS
Daniel Moreno-Páramo
EYELID, CONJUNTIVA & ORBIT
Program
64
Miscellaneous and unknown
(Moderators: M. Giblin, A. Singh)
124 RF39
MYSTERY CASE
Masood Naseripour
158 RF40
A PATIENT WITH MULTIPLE CANCER HISTORY
Helen K. Li
110 RF41
MULTIPLE OCULAR VASCULAR ANOMALIES IN A
CHILD
Mandeep S. Sagoo
1938 RF42
MYSTERY CASE
M. Ashwin Reddy
1834 RF43
MYSTERY CASE
Edoardo Midena
1255 RF44
MYSTERY CASE
Michael E. Giblin
1309 RF45
METASTATIC CHOROIDAL PARAGANGLIOMA
Ann Schalenbourg
67 RF46
MYSTERY CASE
Sara Lally
68 RF47
MYSTERY CASE
Hakan Demirci

Morning
8.30-9.00 Poster presentations ECOp101-111
(Moderator: S. Frenkel, H. Tsuji)
1849 ECp101
FIVE CASES OF CARCINOMA METASTATIC TO THE
EYELIDS
Hiroya Kashiwagi, Hirohisa Katagiri, Takuya Takagi, Hideki Murata,
Mitsuru Takahashi, Toshiaki Takahashi, Masato Matsuzaki
1516 ECp102
NATURAL KILLER/T-CELL LYMPHOMAS IN OCULAR
LESION
Hideki Tsuji, Megumi Kobayashi, Kengo Takeuchi, Kazuhiro Oshitari,
Keigo Shikishima
2337 ECp103
CONJUNCTIVAL SQUAMOUS CELL CARCINOMA
ARISING IN IMMUNOSUPPRESSED PATIENTS (ORGAN
TRANSPLANT, HUMAN IMMUNODEFICIENCY VIRUS
INFECTION)
Carol L. Shields, Aparna Ramasubramanian, Phoebe L. Mellen, Jerry A.
Shields
1822 ECp104
SQUAMOUS NEOPLASIA OF THE OCULAR SURFACE
INVADING THE EYE AND ORBIT: A STUDY OF 30 CASES
Eduardo F. Marback, Ediney Vila Nova Silva, Roberto L. Marback
1150 ECp105
A PROPOSAL FOR A NEW TREATMENT OF ORBITAL
LOW GRADE MALIGNANT LYMPHOMA
Akihiro Kaneko
59 ECp106
ORBITAL EXENTERATION RECONSTRUCTION WITH
RADIAL FOREARM AND
ANTEROLATERAL THIGH FREE FLAP: A
MULTIDISCIPLINARY APPROACH
Sara E. Lally, Carol L. Shields, Joseph Curry, Ryan Heffelfinger, David
Cognetti, Howard Krein, Jerry A. Shields
243 ECp107
A HUGE OCULAR ADNEXAL MALT LYMPHOMA WHICH
AROSE FROM IGG4 RELATED ORBITAL LESION
Koh-ichi Ohshima
223 ECp108
HIGH-RESOLUTION ULTRASONOGRAPHY IN THE
DIAGNOSIS OF ORBITAL MALIGNANT LYMPHOMA
S.V. Saakyan, A.G. Amiryan, V.R. Alikhanova
EYELID, CONJUNTIVA & ORBIT
Posters
65
1752 ECp109
CONJUNCTIVA MALIGNANT MELANOCYTIC TUMORS.
RECURRENCE AND SURVIVAL RATE IN 15 PATIENTS
FROM CHILE
Maria E. Manquez, Pablo Vigorena, Bruno Nervi, Pablo Zoroquiain,
Jeannie Slater, Arturo Espinoza
66 ECp110
LYMPHOPROLIFERATIVE TUMORS OF THE LACRIMAL
GLAND: ANALYSIS OF CLINICAL FEATURES AND
SYSTEMIC INVOLVEMENT
Hakan Demirci, Shivani Gupta, Carol L. Shields, Jerry A. Shields, Victor M. Elner
224 ECp111
IMMUNOHISTOCHEMICAL AND MOLECULAR STUDY
OF OPTIC PATHWAY GLIOMAS
Charles G. Eberhart, J. Douglas Cameron, Elizabeth J. Rushing, Matthias
Karajannis, Fausto J. Rodriguez
Posters Other Intra-ocular Tumors
12.45-13.15 Poster presentations OTp101-110
(moderator J. Pe’er, D. Gombos)
131 OTp101
PHOTODYNAMIC THERAPY FOR ACQUIRED
ASTROCYTOMA
Cinzia Mazzini, Maria Carla Donati, Giulia Pieretti, Ugo Menchini
530 OTp102
MANAGEMENT OF PERIPAPILLARY HEMANGIOMA IN
PEDIATRIC VHL DISEASE
Prithvi Mruthyunjaya
105 OTp103
MEK Inhibitor-associated serous retinopathy -
clinical features of a new retinal disorder
Scott C.N. Oliver, Raul Velez Montoya, Wells Messersmith, Jeffrey L.
Olson, Naresh Mandava
1450 OTp104
BENIGN TUMORS OF CILIARY BODY IN KOREAN
PATIENTS
Christopher Seungkyu Lee, Hee Jung Kwon, Hyae Min Jeon, Min Kim,
Sung Chul Lee
321 OTp105
TREATMENT OF SEROUS MACULAR DETACHMENT
ASSOCIATED WITH CIRCUMSCRIBED CHOROIDAL
HEMANGIOMA
Sung Chul Lee, Hee Jung Kwon, Min Kim, Christopher Seungkyu Lee

2132 OTp106
INTRAOCULAR RELAPSE OF MULTIPLE MYELOMA
RESPONSIVE TO BORTEZOMIB
Tero Kivelä
1923 OTp107
TUBEROUS SCLEROSIS COMPLEX: CHARACTERIZA-
TION OF OCULAR MANIFESTATIONS AND CORRELA-
TIONS WITH SYSTEMIC DISEASE
M.E. Turell, E.I. Traboulsi, A. Gupta , A.D. Singh
248 OTp108
INTERLEUKIN LEVELS IN AQUEOUS OF UNTREATED
AND TREATED EYES WITH VITREORETINAL LYMPHO-
MA
Jose S. Pulido, Joseph Balsanek, Brian Peters, Melissa Snyder
304 OTp109
INTRAVITREAL RITUXIMAB FOR PRIMARY INTRAOCU-
LAR LYMPHOMA (PIOL)
V. Kakkassery, G. Willerding, K. Jahnke, A. Korfel, U. Pleyer, N. Stübiger,
A.M. Joussen
EYELID, CONJUNTIVA & ORBIT
Posters
66

1621 EC1
EVALUATION OF THE “HEDGEHOG” SIGNALLING
PATHWAYS (SHH, PTCH-1, GLI-1 LEVELS) IN
SQUAMOUS AND BASAL CELL CARCINOMAS OF THE
YYELIDS AND CONJUNCTIVA
Hayyam Kiratli1, MD, Ali Rıza Cenk Çelebi1, MD, Figen Söylemezoğlu2, MD
(hkiratli@hacettepe.edu.tr)
1. Ocular Oncology Service, Department of Ophthalmology (and Çelebi)
and 2. Department of Pathology, Hacettepe University School of
Medicine, Ankara, Turkey.
Purpose. To assess the role of hedgehog signalling pathways in the
carcinogenesis of eyelid skin and conjunctival epithelial malignant tumors.
Methods. Shh, the major secreted morphogen protein of the pathway,
Ptch-1, its transmembrane receptor and Gli-1, the target gene involved
in stem cell proliferation were evaluated in paraffin-embedded tissues
using immunohistochemical stainings. The study was conducted on the
specimens of 41 patients with eyelid basal cell carcinoma, 22 with eyelid
and conjunctival squamous cell carcinoma and 12 with conjunctival
intraepithelial neoplasia. For each specimen, the percentage (50%) and the intensity of stainings (graded between 0 to 3) were
calculated and the scores obtained by the multiplication of the two values
were analyzed using Kruskall-Wallis test.
Results. The Shh and Ptch-1 expressions were statistically significantly
lower in the basal cell carcinoma group. In the conjunctival squamous cell
carcinoma group, the Ptch-1 score was 0 in 25% of specimens and Gli-1
score was 3 in 66% of cases. In the conjunctival intraepithelial neoplasia
group, the Ptch-1 score was >2 in 66% of specimens and the Gli-1 score
was 3 in 87.5% of cases.
Conclusions. Ptch-1 mutations may contribute to the development of eyelid
basal cell carcinoma. Alterations in the hedgehog signaling pathways may
lead to transformation of the conjunctival intraepithelial neoplasia into
invasive squamous cell carcinoma.
Financial disclosure. None
1246 EC2
MARGIN CONTROL IN EYELID TUMOR EXCISION: IS
FROZEN SECTION OPTIMAL?
David T.L. Liu, Kelvin K.L. Chong, Gary M.K. Tse, N.M. Luk, Dennis S.C.
Lam (david_tlliu@yahoo.com)
Department of Ophthalmology & Visual Sciences, the Chinese University
of Hong Kong; Department of Anatomical & Cellular Pathology, the
Chinese University of Hong Kong; Dermatology Institute, Department of
Medicine & Therapeutics, the Chinese University of Hong Kong
Purpose. To report the surgical outcomes of periocular basal cell
carcinoma (BCC) patients managed with quick Mohs micrographic
surgery (MMS)
Methods. Prospective series of periocular BCC were enrolled and
managed by quick MMS, which is a simple technique of collapsing
thickness of the specimen, single sectioning and one tissue-level
histopathological examination.
Results. Six female and one male, with age ranged from 58 to 88 were
recruited. Median operation time was 30 minutes. Five patients were
free from postoperative complication. At 18 months, no patient showed
sign of recurrence.
EYELID, CONJUNTIVA & ORBIT
Abstracts
67
Conclusions. In the strike for maximal tumor clearance and aesthetic
preservation, Quick Mohs micrographic surgery may be an express
surrogate for the labor-intensive conventional MMS in the management
of periocular BCC.
Financial disclosure. None
1407 EC3
RADIOTHERAPY FOR MANAGEMENT OF MEDIAL
CANTHAL BASAL CELL CARCINOMA (BCC)
Hatem Krema, Caroline Chung, Evelyn Herrmann, Rand Simpson, David
Payne, Normand Laperriere
(htmkrm19@yahoo.com)
Ocular Oncology and Radiation Oncology Department, Princess Margaret
Hospital/ UHN Toronto, Ontario, Canada
Purpose. To report the efficacy and toxicity of fractionated external
beam radiotherapy for management of medial canthal BCC.
Methods. Retrospective case series analysis. We included consecutive
patients with medial canthal BCC treated with orthovoltage radiotherapy
using direct apposition technique. Patients were included whether
radiotherapy was delivered as a primary treatment or for recurrent
cases after surgical excision, between 1998 and 2009. Kaplan-Meier
estimates were used to calculate actuarial rates.
Results. Included in this study were 92 patients with a median follow-up
of 70 months. The median dose was 35 Gys and the median fraction dose
was 7 Gys. The local control rate was 96% for BCCs treated primarily by
radiotherapy, and 78% for recurrent BCCs after prior surgery. There were
transient post radiotherapy complications in 72% of patients. Chronic
epiphora was present in 19% and chronic dry eye symptoms in 15 %.
Conclusions. Radiotherapy provides an alternative management to
surgical excision and major reconstruction procedures for medial
canthal BCC, with manageable post irradiation side effects.
Financial disclosure. None
1756 EC4
DOES THE SIZE OF EYELID SEBACEOUS CARCINOMA
CORRELATE WITH NODAL METASTASIS AND
SURVIVAL?
Bita Esmaeli1, Qasiem Nasser 1, Hilda Cruz1, Melissa Felman1, Carla L.
Warneke4, Doina Ivan2.3 (besmaeli@mdanderson.org)
1. Section of Ophthalmology, Department of Head and Neck Surgery,
2. Department of Pathology,
3. Department of Dermatology,
4. Department of Biostatistics
The University of Texas MD Anderson Cancer Center, Houston, Texas.
Purpose. To evaluate whether the American Joint Committee on Cancer
(AJCC) 7th edition T designation for eyelid carcinomas correlates with
lymph node metastasis, distant metastasis, and survival in patients with
sebaceous carcinoma of eyelid.
Methods. The records of 50 consecutive patients who were treated by
the principal investigator (BE) for a diagnosis of sebaceous carcinoma
of eyelid between May 1999 and August 2010 were reviewed. Each
patient was staged using the AJCC 7th edition TNM (Tumor, Nodal
status, Distant Metastasis) criteria based on clinical, pathologic, and
radiographic data.

Results. The study included 37 women and 13 men with a median age
of 68.5 years (range: 44 to 86 years). There were 44 whites, 5 hispanics,
and 1 Asian. Tumor location was in the upper eyelid in 28 patients, in the
lower eyelid in 15 patients, and involved both upper and lower eyelids in
7 patients. AJCC 7th edition TNM designations were: TxN0M0,7 patients
(pts); T1N0M0,4 pts.;T2aN0M0,12 pts.;T2bN0M0,11 pts.; T2bN1M0, 2
pts; T2bN1M1,1 patient (pt.);T3aN0M0,2 pt.; T3aN1M0,5 pts.; T3bN0M0,
1 pt; T3bN1M0,1 pt; T3bN0M1,2 pt.; T4N0M0,1 pt.; and T4N0M1,1pt.
Regional lymph node metastasis was seen in 9 patients (18%). The
TNM designation for these 9 patients at last contact were: T2bN1M0
(2 pts), T2bN1M1 (1pt.), T3aN1M0 (5 pts.), T3bN1M0 (1 pt). Presence
of lymph node metastasis was significantly associated with T-stage
at presentation (P = 0.0079). No tumors less than T2b had nodal
metastasis. No tumors less than 9 mm in greatest dimension had nodal
metastasis. Distant metastasis was documentable in 4 patients and
death from disease occurred in 5 patients (10%). The TNM designations
for these 5 patients were: T2bN1M1 (1pt.), T3bN0M1 (2pts.), T4N0M0 (1
pt), and T4N0M1 (1pt). No tumors less than 12 mm in greatest dimension
were associated with distant metastasis or death. Disease-specific
survival (DSS) was significantly associated with T-stage of the primary
tumor (P = 0.0009). DSS was poorer among patients who presented
with a tumor > T3a using the 2-category t-stage (P = 0.035).
Conclusions. The 7th edition AJCC criteria for eyelid carcinomas correlate
with outcomes for sebaceous carcinoma of eyelid. Tumor size correlates
with regional lymph node metastasis with tumors >10 mm in greatest
dimension being at risk. Sebaceous carcinomas > T3a are associated
with poorer survival.
Financial disclosure. None
2244 EC5
LYMPHOMA OF THE EYELIDS – A NATION-BASED STUDY
Steffen Heegaard1,3, Peter K. Rasmussen 1, Elisabeth Ralfkiaer2, Lene
D. Sjö2 and Jan U. Prause1 (sthe@sund.ku.dk)
1. Department of Neuroscience and Pharmacology, Eye Pathology
Institute, University of Copenhagen, Denmark
2. Department of Pathology, Copenhagen University Hospital, Denmark
3. Department of Ophthalmology, University of Copenhagen, Glostrup Hospital,
Denmark
Purpose. To characterize the clinicopathological features of lymphoma
of the eyelids
Methods. All cases of eyelid lymphoma between 1980 and 2008 were
retrieved from the Danish Registry of Pathology. Histological specimens
were re-evaluated using a panel of monoclonal antibodies. Clinical files
from all patients with confirmed lymphoma were collected.
Results. Twenty-five patients with lymphoma of the eyelids were
identified. Thirteen of the patients were males and the median age was
66 years (range 31 to 83 years). The distribution of lymphoma subtypes
were: extranodal marginal zone lymphoma (EMZL) 44% (11), mantle
cell lymphoma (MCL) 20% (5), follicular lymphoma (FL) 12% (3), diffuse
large B-cell lymphoma (DLBCL) 8% (2), peripheral T-cell lymphoma,
unspecified (PTCL, NOS) 8% (2), chronic lymphocytic leukemia/ small
lymphatic lymphoma (CLL/SLL) 4% (1) and anaplastic large T-cell
lymphoma (T-ALCL) 4% (1).
Fourteen patients (56%) presented with Stage I/II lymphoma. Two
patients (8%) had Stage III lymphoma and nine patients (36%) presented
with Stage IV lymphoma.
The 5-year overall survival rate (OS) was 42%. The patients with lowgrade
lymphoma subtypes (EMZL; FL; PTCL, NOS; CLL/SLL) had a
EYELID, CONJUNTIVA & ORBIT
Abstracts
68
significantly better 3-year OS rate (77%) than patients with high-grade
lymphoma (MCL; DLBCL; T-ALCL) (3-year OS rate, 21%).
Conclusions. Lymphoma of the eyelids is relatively rare and is mainly
prevalent in elderly patients. Most patients had unilateral involvement.
The occurrence of MCL was relatively high compared to the distribution of
lymphoma subtypes of the orbit. The prognosis for the whole population
was relatively poor, however, patients with low-grade lymphoma had a
significantly better survival.
Financial disclosure. None
143 EC6
DIAGNOSIS AND FOLLOW-UP OF OCULAR SURFACE
SQUAMOUS NEOPLASIA BASED ON NON-INVASIVE
IN VIVO BIOPSY USING CONFOCAL MICROSCOPY
J. Oscar Croxatto, MD, Carolina Gentile, MD (juan.croxatto@gmail.com)
Oncology Unit, Hospital Italiano de Buenos Aires, Fundación
Oftalmológica Argentina J. Malbran and Laboratorios Pörtner, Buenos
Aires, Argentina.
Purpose. To analyze the tissue findings, diagnosis and follow-up of
corneal and conjunctival epithelial neoplasia in a large series of patients
examined with in vivo confocal microscopy.
Methods. Fifty-five patients with a presumed diagnosis of corneal
and conjunctival epithelial neoplasia underwent examination with
in vivo confocal microscopy (CCFM) (Rostock corneal module/HRTII,
Heildelberg). The CCFM findings were compared with histology and
cytology in those cases which underwent surgical excision or ocular
surface impression cytology. The main outcome measures were CCFM
findings, diagnosis, resolution after therapy, and histopathologic and
cytologic findings.
Results. The CCFM diagnoses were cornea and conjunctival
intraepithelial neoplasia (38 cases), early invasive epithelial neoplasia
(4 cases), intraepithelial neoplasia and pterygium (3 cases), stem cell
deficiency (3 cases), UV-related keratosis (4 cases), pterygium without
epithelial atypia (2 cases), and squamous cell carcinoma (1 case).
CCFM findings showed a precise correlation with histopathological and
cytological specimens. The post-medication examination procedure
could confirm resolution ad integrum in patients treated with mitomycin
C, and periodic evaluation until complete resolution in those patients
receiving interferon alfa2-b. Subclinical recurrences were identified in
3 cases.
Conclusions. CCFM provides an excellent non-invasive in vivo image
method to confirm the diagnosis of cornea and conjunctival neoplasia, to
detect early invasion, to guide medical treatment in patients undergoing
topical therapy, and to differentiate limbal and corneal simulating diseases.
Financial disclosure. None
1459 EC7
OCULAR SURFACE SQUAMOUS NEOPLASIA IN A
PATIENT WITH HIV INFECTION: REGRESSION AFTER
RESTARTING ANTIRETROVIRAL THERAPY WITH
DEVELOPMENT OF SPECIFIC T-CELLS
M. Marinkovic, A. Rengifo Coolman, M.J.P. Schoenmaekers-Welters, S.
van der Burg, M.J. Jager, G.P.M. Luyten (m.marinkovic@yahoo.com)
Dept. of Ophthalmology and Dept. of Oncology, Leiden University
Medical Centre, Leiden , The Netherlands

Purpose. Ocular surface squamous neoplasia (OSSN) refers to
precancerous and cancerous lesions of the conjunctiva ranging form
conjuctival intraepithelial neoplasia (CIN) to an invasive tumor with
destruction of the orbital tissues. OSSN used to affect elderly men, yet
this pattern has changed. In Africa the incidence is rising in younger
persons, mostly women around 35 years of age. This increasing
prevalence is probably due to HIV infection and HPV co-infections.
Methods. Clinical and immunological analysis including testing of
specific T cell interferon-gamma immune responses, and tissue HPV
type analysis.
Results. A 50 year-old female presented with complaints of chronical
blepharoconjunctivitis which had existed for a couple of years. She was
HIV positive and used antiretroviral medication infrequently. An eye
examination revealed extensive leukoplakia of the palpebral and bulbar
conjunctiva and cornea in the left eye. As a biopsy revealed squamous
cell carcinoma with infiltration of the tarsal and bulbar conjunctiva of
the left eye, exenteration of the left orbit was planned.
When admitted to the hospital after a patients delay of a couple of
months, the lesion of the conjunctival lesion had almost disappeared.
The patient mentioned that she had restarted to take her HAART
medication conscientiously.
Earlier biopsies showed the presence of HPV 16, and at the time of tumor
regression, anti-HPV16 T-cell responses were found to be present.
Conclusions. Meticulous use of antiretroviral medication may cause
regression of OSSN. It is unclear whether the medication targets the
underlying HIV or HPV infection, or indirectly led to healing by allowing
the recurrence of an anti-HPV16 T cell immune response. The lack of
compliance in the use of therapy could be added to the list of risk factors
for OSSN. Further research is needed to confirm this suggestion.
Financial disclosure. None
12 EC8
RUTHENIUM PLAQUE THERAPY IN THE MANAGEMENT
OF CONJUNCTIVAL SQUAMOUS CELL CARCINOMA
INVADING THE EYE
Priscilla L. Ballalai, Virginia L. Torres, Roberto Segretto (pbbordon@
terra.com.br)
Ocular Oncology Section - Federal University of Sao Paulo
Radiotherapy - Federal University of Sao Paulo
Purpose. To describe the outcome of patients with conjunctival
squamous cell carcinoma invading the eye treated with Ruthenium
(Ru106) plaque therapy.
Methods. Retrospective, non-comparative review of charts of patients
referred to the University and private office with conjunctival squamous
cell carcinoma (SCC) invading the eye, from January 2001 to July 2011.
Patients with focal invasions at the cornea, sclera or iris and ciliary body
were included. Patients with orbital infiltrations or extensive intraocular
infiltrations were excluded. All patients were submitted to Ru 106 plaque
therapy, and the treatment dose was 50 Gy. The duration of the treatment
was calculated according to the UBM measurements with a 1 mm safety
margin. The patients were followed with a complete ophthalmological
examination and systemic evaluation after the treatment.
Results. Twenty patients with conjunctival SCC invading the eye were
seen by the authors from January 2001 to July 2011. Six of them were
eligible for salvage therapy with ruthenium plaque therapy. One patient
was HIV positive. The mean age was 58 yo (range 29-82 yo), 4 were
females and 2 males. The mean follow up of was 31,5 mo (range 6 -108
EYELID, CONJUNTIVA & ORBIT
Abstracts
69
mo). All of them had been treated previously with surgery or surgery and
topical chemotherapy (Mitomycin C and/or Interferon). The mean number
of surgeries was 2,1 (range 1-4). The pathological evaluation confirmed
the diagnosis of SCC in all patients. Infiltration of the corneal stroma
was observed in 3 patients, deep scleral infiltration in 4 patients and
iris and ciliary body infiltration in 1 patient. All patients had regression
of the tumor after the treatment without recurrence in the treatment
site or elsewhere. None of them presented distant metastasis. The only
complication observed with this treatment was cataract, in 3 patients.
Conclusions. Ruthenium plaque therapy is safe and effective in the
management of eyes with focal infiltrations by conjunctival squamous
cell carcinoma.
Financial disclosure. None
1059 EC9
INVASIVE SQUAMOUS CELL CARCINOMA OF THE
CONJUNCTIVA TREATED BY PROTONS
W. Sauerwein1, J. Herault2, P. Chauvel2, H. Westekemper3, R.
Darawsha3, B. Zimmermann1, C. Maschi4, A. Wittig5, L. Brualla1, J.-P.
Caujolle4 (w.sauerwein@uni-due.de)
1. University Duisburg- Essen, University Hospital Essen, Department of
Radiation Oncology, Essen, Germany
2. Cyclotron Biomédicale, Centre Antoine-Lacassagne, Nice, France
3. University Duisburg- Essen, University Hospital Essen, Department of
Ophthalmology, Essen, Germany
4. Service d’Ophtalmologie, CHU Saint Roch, Nice, France
5. Klinikum der Philipps-Universität Marburg, Department of Radiation
Oncology Marburg, Germany
Purpose. Squamous cell carcinoma (SCC) of the conjunctiva is an
extremely rare disease. The disease is suspected to be associated
with atrophic dermatitis and HIV in young women. After local excision
the tumor often recurs instead of adjuvant therapies such as cryocoagulation,
local chemotherapy or radiotherapy. Conventional external
beam radiotherapy with electrons or photons is not conducive as the
dose needed to control the tumor will destroy the eye. A sufficient dose
can only be delivered by brachytherapy if the tumor is localized on the
eyeball and if the target volume can be covered by a radioactive plaque.
Therefore, exenteration is often the only curative approach. Protons
might offer an alternative as they can be collimated and modulated to
cover a complex area of the conjunctiva including fornix and tarsus while
protecting the inner part of the eye and radiosensitive structures in the
neighbourhood.
Methods. The irradiation technique was initially developed to treat
melanoma of the conjunctiva. The target volume to be considered is
large and individually shaped. It includes often more than half of the total
surface of the conjunctiva, with the goal to avoid further relapses at the
margin of the treated volume. Because of the large volume, the number of
fractions has been extended as compared to conventional proton therapy of
uveal melanoma. The dose applied is given in 6 fractions of 5.2 Gy (36 Gy
RBE) followed by a boost of 2 fractions of 7 Gy (16 Gy RBE). The technical
principles of the treatment are as follows: - the clips are inserted on the eye
for localization of the target volume and positioning. A bolus is set-up on the
lid to give a homogeneous flat entrance for the proton beam collimated to
the size of the tumor, - the beam passes through a semi-spherical acrylic
glass compensator to adapt the range of the proton beam to the shape
the inner sclera and let the protons irradiate the thickness of sclera and
conjunctiva. The compensator is individually customized from an acrylic
glass block by a computerized milling machine. The resulting dose distribution is

homogeneous and gives the whole dose to the bulbar and tarsal conjunctiva.
Results. We report results from 24 HIV negative patients who received
proton radiotherapy in Nice between 2001 and 2010. Mean follow up is
40 months. None of the patients died for metastases. Tumor control was
achieved for 19 patients (79%). 5 recurrent tumors were observed 2 – 8
years after the irradiation. None of them occurred inside the irradiated
volume. In one case, a second proton irradiation could control the
disease. Exenteration was performed in the other cases.
Conclusion. Proton irradiation offers the possibility to successfully
treat SCC of the conjunctiva with acceptable side effects and can be
considered as an alternative to traditional treatments.
Financial disclosure: None
1505 EC10
PROSPECTIVE STUDY OF SENTINEL LYMPH NODE BI-
OPSY FOR CONJUNCTIVAL MALIGNANT MELANOMA
Victoria M.L. Cohen, Maria Tsimpida, Norbert Avril, John L. Hungerford,
Grahem Moir (victoria.lendrum@gmail.com)
Ocular Oncology Service. St Bartholomew’s and Moorfields eye Hospital,
London UK
Department of Nuclear Medicine, St Bartholomew’s Hospital, London UK
Department of Plastic Surgery, St Bartholomew’s Hospital London UK
Purpose. To report our experience with sentinel lymph node biopsy for
staging patients with conjunctival malignant melanoma.
Methods. All patients with conjunctival malignant melanoma who
had sentinel lymph node biopsy at St. Bartholomew’s Hospital from
May 2008 to May 2011 were included in this study. The main outcome
measures were the incidence of sentinel lymph node positivity, the
procedure-related complications and the metastasis free survival rate.
Results. In 3 years, 23 patients met the selection criteria for sentinel
lymph node biopsy. 4 patients declined and 19 patients were consented
for the procedure. Technetium-99m failed to identify the lymph nodes
in 4 of the 19 patients (21%). Of the remaining 15 patients, 2 were
found to have subclinical micrometastasis in regional lymph nodes. No
false-negative events were observed. Complications of the procedure
included transient blue staining of the epibulbar surface in 3 patients
and transient facial nerve palsy in 1 patient.
Conclusions. Sentinel lymph node biopsy is a safe procedure with
minimal complications. It should be considered for the staging of
conjunctival melanoma, especially non-limbal melanoma or conjunctival
melanoma more than 2mm thick.
Financial disclosure. None
2202 EC11
EXENTERATION FOR CONJUNCTIVAL MELANOMA IN
LIVERPOOL: 1993-2010
Nihal Kenawy, Sarah E. Coupland, Bertil E. Damato (nkenawy@liverpool.ac.uk)
Liverpool Ocular Oncology Centre, Royal Liverpool University Hospital,
Prescot Street, Liverpool, UK
Purpose. To report the incidence and causes of exenteration in patients
with conjunctival melanoma treated at the Liverpool Ocular Oncology
Centre (LOOC) between 1993 and 2010.
Methods. Database review of all patients with conjunctival melanocytic
disease treated in LOOC between 1993 and 2010. Patients treated
with exenteration were identified and the case notes were reviewed to
EYELID, CONJUNTIVA & ORBIT
Abstracts
70
identify the reasons for exenteration.
Results. We treated 99 patients (52 females, 47 males; median age 62
years, range 24-95) for conjunctival melanoma between 1993 and 2010.
Four patients required exenteration. Two were males and two females. At
initial presentation, three patients had biopsy-proven invasive melanoma
and one patient had melanoma in situ (C-MIN score 9). One patient had
primary exenteration because of advanced disease at presentation and
three had secondary exenteration because of uncontrollable disease
and/or pain. One of the three patients commenced treatment in 1995
prior to our updated policy of brachytherapy for invasive melanoma
and the condition was uncontrolled with multiple sessions of topical
mitomycin C and cryotherapy. The caruncle and lacrimal passages
were involved in the two other patients with invasive melanoma, both
of whom had undergone excision biopsy with inadequate clearance
prior to referral to LOOC. Both patients had received adjunctive orbital
radiotherapy but one developed distant metastasis.
Conclusions. Exenteration for conjunctival melanoma is rare in patients
undergoing primary treatment with adjunctive brachytherapy and topical
chemotherapy. The main causes of exenteration were uncontrollable
disease and pain.
Financial disclosure. None
2311 EC12
TEENAGE ELEVATED CONJUNCTIVAL LESIONS IN THE
PLICAL AREA – LYMPHOMA OR REACTIVE LYMPHOID
HYPERPLASIA?
Shahar Frenkel, MD, PhD, Jacob Pe’er, MD(shahar.frenkel@gmail.com)
Specialized Ocular Oncology Service, Department of Ophthalmology,
Hadassah - Hebrew University Medical Center, Jerusalem, Israel
Purpose. Ocular adnexal lymphoma (OAL) comprises 2.5% of extranodal
non-Hodgkin lymphomas, and is most commonly a marginal zone
lymphoma of mucosa-associated lymphoid tissue (MALT). The mean
age at diagnosis is 63, with only 1.4% of cases younger than 21 years
of age. We describe a series of cases with a similar clinical presentation
but with a different final diagnosis in teenage patients.
Methods. Retrospective chart review of patients with a clinical
presentation of indicative of teenage OAL, presenting to our clinic in the
past 5 years.
Results. Five teenagers aged 14-17, all male, presented with bilateral
large elevated pink conjunctival masses immediately adjacent to or even
connected to the plica. None of them complained of allergic symptoms
on presentation, but 3 (cases 3-5) confirmed symptoms on questioning.
Cases 1-3 did not respond to anti-allergic medications and underwent
excisional biopsies that revealed MALT lymphoma in cases 1 and 2, and
reactive lymphoid hyperplasia in case 3. Cases 4 and 5 were treated
with anti-allergic medication with resolution of the findings, and were
clinically diagnoses as reactive lymphoid hyperplasia.
Conclusions. OAL is rare in the pediatric group, but reactive lymphoid
hyperplasia can present in teenage (male) patients with large plical
masses that can resemble OAL.
Financial disclosure. None
1933 OT1
CLINICAL SURVEY OF 3680 SOLID AND CYSTIC TU-
MORS OF THE IRIS
Carlos Bianciotto, Carol L Shields, Swarupa Kancherla, Mayerling
Suriano, Margaret V. Shields, Priya Sharma, Jinali Patel, Jerry A. Shields

(cargusale@yahoo.com)
Oncology Service, Wills Eye Institute, Philadelphia
Purpose. To report the spectrum of iris lesions from a single ocular
oncology center
Methods. Retrospective review of medical records
Results. Of 3451 patients, the mean age at presentation was 48 years
(median 50 years, range 2 week to 95 years). There were 1423 males
(41%) and 2028 females (59%). Of 3680 lesions, 2907 (79%) were
benign and 773 (21%) were malignant, while 768 were cystic (21%)
and 2912 (79%) solid. Specific diagnoses included nevus (41%), iris
pigment epithelium (IPE) cyst (18%), melanoma (18%), freckle (3%),
Lisch nodules (2%), melanocytoma (2%), melanocytosis (2%), and
other (15%). Lesions were located in the iris stroma (80%) and IPE
(20%). Most common diagnostic categories within solid lesions were
melanocytic (68%), non-melanocytic (11%), vascular (2%), metastasis
(2%) and pseudotumors (5%).
Conclusions. Of 3680 iris lesions, 79% were benign; and the most
common diagnosis included nevus (41%), IPE cyst (18%), melanoma
(18%), and freckle (3%).
Financial disclosure. None
111 OT2
CLINICAL AND OCT FINDINGS ON SYMPTOMATIC
MACULAR NAEVI TREATED WITH INTRAVITREAL
ANTI-VEGF
Sonia A. Callejo, Mikael Sebag, Marc Blouin, Christine Corriveau
(guilleyso@hotmail.com)
Centre Hospitalier de l’Universite de Montreal CHUM Canada
Purpose. To report the clinical and OCT findings of patients with
symptomatic macular naevi treated with intraocular anti-VEGF.
Methods. Retrospective small case series of 8 patients diagnosed
with macular choroidal naevus with impared visual acuity treated
with intravitreal anti-VEGF. Clinical information, serial color
fundus photography, FA, OCT findings and ultrasonographic tumor
measurements were reviewed and compared before and after anti-VEGF
treatment.
Results. 8 patients: 4 males and 4 females. Mean age at presentation:
63 years (range 49 to 54). Mean follow up: 3 ½ years (range 7 months-9
1/2 years). Mean number of anti-VEGF injections: 7.5 (range: 1 to 17).
The largest naevus basal diameter was 7.3mm and the median thickness
was 2.1mm. No ultrasonographic evidence of tumor enlargement or
transformation into melanoma was documented following treatments.
Large fluctuations in VA were recorded prior to anti-VEGF injections.
Reduction in the degree of fluctuation was noticed during anti-VEGF
treatments. Anti-VEGF treatment was associated with resolution or
decreased of exudative retinal detachment, retinal thickness, cystic
retinal changes (4 cases), subretinal fluid (6 cases), pigmented
epithelium detachment (PED) (3 cases) and subretinal/intraretinal
blood associated (1 case) or not (2 cases) to the presence of a choroidal
endovascular membrane.
Conclusions. Although a larger study is needed, the use of intraocular
anti-VEGF treatment of symptomatic macular naevi is promising based
on the improvement of clinical and OCT parameters as well as the
stability of tumor measurements.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstracts
71
1340 OT3
RISK FACTORS FOR GROWTH OF POSTERIOR UVEAL
MELANOCYTIC LESIONS WITH THICKNESS GREATER
THAN 2 MM IN 161 CONSECUTIVE PATIENTS
Patrick De Potter, Audrey Noel, Jacques Jamart
(patrick.depotter@uclouvain.be)
Ocular Oncology Unit, Centre du Cancer, Cliniques Universitaires St Luc,
Brussels, Belgium.
Centre de Biostatistique et de Documentation médicale, Cliniques
Universitaires de Mont-Godinne, Yvoir, Belgium
Purpose. To better define the clinical and ultrasonographic risk factors
predictive of growth of small melanocytic posterior uveal tumors
presenting with a thickness of 2 mm or more as the only clinical suspicious
factor predictive of tumor growth at first visit.
Methods. Non comparative observational cases series including 161
patients with suspicious choroidal or cilio-choroidal tumors measuring
2 mm or more in thickness and no other clinical factors predicting of
growth such as orange pigment, subretinal fluid, tumor margin touching
disc, or tumor-related visual symptoms at initial visit. Those 161 patients
were followed since October 1997 to document growth prior to treatment.
Kaplan-Meier analysis was used to assess time to tumor growth and Cox
proportional hazards regressions evaluated factors predictive of tumor
growth
Results. The mean age at diagnosis was 65 years old. The mean largest
tumor diameter was 9 mm (range, 3 to 16 mm) and tumor thickness 2,6
mm (range, 2 to 5,6 mm). The mean distance to the disc was 5,7 mm
(range, 0,5 to 15 mm) and to the fovea 5,2 mm (range, 0 to 16 mm). Of
the 161 small melanocytic lesions, 31 (19%) demonstrated growth after a
mean follow-up of 32 months (range, 6 to 116 months). One patient (0.6%)
developed systemic metastasis. The factors predictive of growth included
greater tumor diameter, greater tumor thickness, lack of drusen, and
acoustic hollowness. The global cumulative risks for tumor growth were
4% at one year, 17% at 3 years, 22% at 5 years, and 27% at 10 years.
Conclusions. Among posterior uveal melanocytic lesions with tumor
thickness greater than 2.0 mm as the only clinical factor predictive of
growth at initial visit, those with greater diameter, greater thickness,
acoustic hollowness and lack of overlying drusen carried a significant risk
for growth and should be monitored with regular surveillance.
Financial disclosure. None
6 OT4
THE EXPANDING SPECTRUM OF RETINAL VASOPRO-
LIFERATIVE TUMOURS
Jerry A. Shields MD, David Reichstein MD, Arman Mashayekhi MD, Carol
L. Shields MD (jerryshields@comcast.net)
Wills Eye Institute
Purpose. Retinal vasoproliferative tumor (RVPT) was originally
described as an idiopathic fundus lesion with distinct clinical features.
Subsequently, it found to be associated with a variety of ocular and
systemic conditions and was subdivided into primary and secondary types.
Methods. Review of lesions coded as retinal RVPT on the Oncology
Service of Wills Eye Institute and literature search for causes of
secondary retinal RVPTs
Results. The secondary form of RVPT has now been associated with a

variety of ophthalmic disorders such as intermediate uveitis, retinitis
pigmentosa, choroidal coloboma, longstanding retinal detachment,
Coats’ disease, retinopathy of prematurity, familial exudative
vitreoretinopathy, neurofibromatosis type 1, x-linked retinoschisis and
other disorders.
Conclusions. Lesions similar to idiopathic RVPT have now been recognized
with an array of well known fundus entities and ophthalmologists should
be aware of these associations.
Financial disclosure. None
518 OT5
DIFFUSE CHOROIDAL HEMANGIOMA: CLINICAL MANI-
FESTATIONS AND VISION OUTCOME IN 60 PATIENTS
H.K. Li1,2,3, C.L. Shields1, N. Ni1, J. Iturralde1, J.A. Shields1 (hlimed@
mac.com)
1. Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA.
2. The Methodist Hospital / Weil Cornell Medical College, Houston, TX
3. The University of Texas Health Science Houston, Houston, TX
Purpose. Evaluation of diffuse choroidal hemangioma clinical features
and vision outcome.
Methods. Retrospective clinic-based study.
Results. 73 eyes of 60 patients with diffuse choroidal hemangioma
were diagnosed on the Oncology Service at Wills Eye Hospital between
1969 and 2010. Median age was 15 years (range, 21 to 88 years). Eye
history included glaucoma (66%), amblyopia (29%) and strabismus
(14%). Associated clinical findings included facial nevus flammeus
(95%) and brain angioma (28%). Angioma involved both upper and
lower lids (68%), upper lid only (29%), lower lid only (4%), conjunctiva
(53%) and episclera (67%). Diffuse choridal hemangioma involved
both eyes (22%), right eye only (32%), and left eye only (47%). Median
thickness was 4.3mm (range, 1.3 to 11mm). Other features included
circumscribed nodule (34%), venous dilatation and tortuosity (22%),
and subretinal fluid (52%).
Median follow-up of 57 eyes of 47 patients was 5 years (range, 0.3 to
24.3 years). Fifteen eyes (21%) had subretinal fluid management prior
to referral and 29 eyes (51%) after referral. Median age of patients
diagnosed with subretinal fluid was 13 years (range 3.5 to 48 years).
At final visit, 8 (14%) eyes had improved visual acuity, 43 (75%) were
stable, and 6 (11%) had worsened. Final visual acuity was ≤ 20/200
in 29 (51%) eyes, 20/50-20/200 in 9 (16%) eyes, and 20/20-20/40 in
19 (33%) eyes. At final visit, eyes with subretinal fluid at initial visit
showed poor vision of ≤ 20/200 in 21 (62%) eyes compared to those
without subretinal fluid at initial visit in 8 (35%) eyes.
Conclusions. Diffuse choroidal hemangioma commonly produces
vision-threatening subretinal fluid. Eyes with diffuse choroidal
hemangioma should be monitored closely for detection and treatment
of subretinal fluid.
Financial disclosure. None
504 OT6
SD-OCT AND AUTOFLUORESCENCE CHARACTER-
ISTICS OF CANCER-ASSOCIATED AUTOIMMUNE
RETINOPATHY
Prithvi Mruthyunjaya MD and Kathryn L. Pepple MD, PhD (mruth001@
mc.duke.edu)
EYELID, CONJUNTIVA & ORBIT
Abstracts
72
Ocular Oncology and Vitreoretinal Surgery Services, Duke Eye Center,
Durham, North Carolina
Purpose. To identify the SD-OCT and fundus autofluorescence (AF)
characteristics found in patients diagnosed with cancer associated
autoimmune retinopathy (c-AIR).
Methods. Retrospective review of patients diagnosed with AIR at the
Duke Eye center. Patients were included that demonstrated bilateral
visual field loss, had abnormal electroretinograms (ERGs), had serum
autoantibodies against retinal antigens demonstrated on western blot,
and had SD-OCT and AF imaging of the fundus obtained at the time of
presentation. Imaging studies were reviewed and the group of patients
with abnormal findings was compared to the normal imaging cohort.
Cancer status and autoantibody profiles were tested for correlation with
abnormal imaging.
Results. 12 patients were identified that met the inclusion criteria.
Average age was 57 +/- 16 years (range 24 to 80), 4 males and 8
females, 7 of 12 had a prior or recent diagnosis of systemic cancer. Ten
of 12 patients were identified with AF abnormalities consisting of central
or paracentral macular hypo-autofluorecence surrounded by hyperautofluorescence.
SD-OCT imaging in these patients was also abnormal
demonstrating peripheral loss of the photoreceptor layer with sparing
of the central macula. The boundary of abnormal AF corresponds to the
boundary of photoreceptor layer loss on SD-OCT. Comparison of the
group of patients with imaging changes to those without did not reveal
a significant correlation of imaging abnormalities with the any specific
autoantibody.
Conclusions. This is the first study to identify both SD-OCT and AF
imaging abnormalities in patients with c-AIR. Vision loss corresponds
with outer retinal dysfunction and RPE loss.
Financial disclosure. None
1801 OT7
NEAR- INFRARED AUTOFLUORESCENCE OF NEUROFI-
BROMATOSIS TYPE 1
Minoru Furuta, Tetuju Sekiryu, Tomohiro Iida (furuta@fmu.ac.jp)
Department of Ophthalmology, Fukushima Medical University School of
Medicine, Fukushima, Japan
Purpose. To determine presence of melanin pigment using near-infrared
(NIR) autofluorescence in patients with choroidal neurofibromatosis
type1 (NF-1).
Methods. This case series includes ten eyes of five patients (one man
and four females) with NF-1. Diagnosis was given under the National
Institute of Health criteria for the diagnosis of NF-1. The patients were
taken complete ophthalmic examination including spectral-domain
optical coherence tomography, fluorescein angiography, indocyanine
green angiography, and NIR autofluorescence (excitation 788
nanometers; emission > 800-900 nanometers) imaging which was
performed using HRA2 (Heidelberg Retina Angiogram 2, Heidelberg
Engineering, Heidelberg, Germany).
Results. Mean age of five patients was 38 year-old, ranging from 18 to
60 year-old. NIR reflectance of posterior ocular fundus revealed highreflective
patchy choroidal lesion in ten of all eyes. NIR autofluorescence
was detected in every lesions observed in NIR reflectance imaging.
Conclusions. NIR autofluorescence originates from oxidized melanin, or
compounds closely associated with melanin in retinal pigment epithelium
and choroid.1) Recent clinical observations reveal that the choroid is one

of the tissues most commonly affected by NF-1. Because the multiple,
bright patchy spots were detected in 100% of cases under NIR reflectance
imaging.2) In NF-1 patients, to prove presence of abnormal pigment
accumulation which may originates from choroidal Schwann cells or
nevoid cells will lead to better understandings of disease.
1) Keilbauer CN, et al. IOVS 2006, 2)Yasunari T, et al. Lancet 2000
Financial disclosure. None
2340 OT8
VASOPROLIFERATIVE LESIONS (VPLS) OF THE RETINA
AND THE OPTIC DISC
Vicktoria Vishnevskia-Dai1, Josef Moisseiev1, Amir Elhalel1, Iris Moroz1 ,
Roth Sigal2, Mordechai Rosner1 (vivida65@gmail.com)
1. Goldschleger Eye Institute, Sheba Medical center, Sackler School of
medicine, Tel-Aviv University Israel.
2. Rabin Medical center, Sackler School of medicine, Tel-Aviv University
Israel.
Purpose. Vasoproliferative lesions (VPLs) of the Retina and the Optic Disc
consist of four rare clinical entities: Retinal capillary hemangioma, other
retinal vasoproliferative lesions, retinal cavernous hemangiom and retinal
arteriovenous communications (Wyburn-Mason syndrome).
Our purpose is to report our experience with the management of patients with VPLs.
Methods. Consecutive case series of 10 patients with VPLs treated at the
Sheba medical center, Tel Aviv University, between the years 2004-2011.
Results. The series included 14 eyes of 10 patients (8 males and 2
females). The average age of the patients was 40.2 years (9-74years). The
follow up time was 4-84 months (mean 29.8 months).
Five of the patients had associated systemic disease. Two patients had
associated ocular condition.
Five eyes needed observation only. Of the eyes that needed intervention
3 were injected with anti VEGF, 3 were treated with cryo-ablation, 3 were
treated with argon laser photocoagulation, one with trans papillary
thermo therapy and 3 eyes needed vitrectomy.
The final visual acuity was 6/6 or better in 8 eyes (57%) 6/12 or better in
11 eyes (78%) and less then 6/60 in 3 eyes (21%).
Conclusions. Vasoproliferative lesions of the retina and the optic disc are
rare ocular pathologies with a wide clinical spectrum of presentation.
Proper systemic workup and patient tailored management can prevent
ocular and systemic morbidity, and result in good visual acuity in most
of the patients.
Financial disclosure. None
2113 OT9
VITREORETINAL SURGERY FOR TRACTIONAL COMPLI-
CATIONS ASSOCIATED WITH JUXTAPAPILLARY COM-
BINED HAMARTOMA OF THE RETINA AND RETINAL
PIGMENT EPITHELIUM
Javier Elizalde (jem25@telefonica.net)
Ocular Oncology Service, Barraquer Institute, Barcelona, SPAIN
Purpose. To describe clinical features of tractional complications
associated with combined hamartoma of the retina and retinal pigment
epithelium (CHRRPE) and to discuss the surgical indications and
technique, and the functional outcome after vitreoretinal surgery.
Methods. Our Ocular Oncology database and the clinical data were
EYELID, CONJUNTIVA & ORBIT
Abstracts
73
reviewed. Microincisional vitreoretinal surgery with 23G instruments
was performed to relieve the tractional component when the patient
complained of progressive visual loss.
Results. Clinical findings indicating retinal traction associated with
CHRRPE include progressive distortion and displacement of the
neighboring retina, stretching of the blood vessels, telangiectasias,
retinal striae, exudation, superficial hemorrhages, retinal pigment
epithelium abnormalities and full-thickness retinal folds. Vitreoretinal
surgery usually improves the anatomical aspect of the retina although
the functional improvement can be limited.
Conclusions. Surgical dissection is partially effective in the treatment
of vitreomacular traction associated with CHRRPE because amblyopia,
long-standing intraretinal abnormalities and chronic photoreceptor
damage usually limit the functional outcome. Early detection and
intervention for cases presenting a recent vision loss are thought to
show more positive results.
Financial disclosure. None
206 OT10
RISK OF RADIATION RETINOPATHY IN PATIENTS WITH
ORBITAL AND OCULAR LYMPHOMA
Megha Kaushik, MBBS(hons) BSci(med)hons1, Jose S. Pulido1, MD
MS MPH, Khushboo K. Agrawal1, MD, Steven E. Schild2, MD, Scott
Stafford2, MD
(megha.ka@gmail.com)
1. Department of Ophthalmology
2. Department of Radiation Oncology
Mayo Clinic
Purpose. The aim of this study is to identify the risk of developing radiation
retinopathy at increasing radiation doses.
Methods. A 40 year retrospective review was performed of patients who
received external beam radiation therapy for ocular/orbital non-Hodgkin’s
lymphoma (NHL).
Results. Sixty-seven patients who had at least one ophthalmic follow up
examination were included in this study. Most patients were diagnosed
with NHL involving the orbit (52%). The patients received external beam
radiation therapy at doses between 1886 to 5400cGy (mean 3033 ± 782
cGy). 12% of patients developed radiation retinopathy with the mean time
to diagnosis of 60 ± 76 months. The mean radiation dose in patients with
retinopathy was 3309 ± 585cGy and the estimated retinal dose (derived by
formulae) was 3087 ± 1030cGy. The incidence of retinopathy increased with
dose. The mean visual acuity of the eyes that received radiation was worse
than the eyes that did not (p=0.027). More patients with post-radiation
retinopathy had comorbid diabetes mellitus type 2 compared to patients
without retinopathy (p=0.015). Other post-radiotherapy ocular findings in
our study included keratitis (7%), dry eyes (39%), and cataract (33%).
Conclusions. Radiation retinopathy is a known complication of therapy for
tumors within the orbit appears related to co-morbidities and dose. When
treating an orbital tumor, one should always use the lowest effective dose
and spare the eye to the greatest extent possible.
Financial disclosure. None
2324 OT11
VITREORETINAL LYMPHOMA TREATED BY INTRAVIT-
REAL INJECTIONS OF METHOTREXATE: 80 EYES OVER
14 YEARS
Jacob Pe’er, MD, Shahar Frenkel, MD, PhD (peer@hadassah.org.il)

Specialized Ocular Oncology Service, Department of Ophthalmology
Hadassah - Hebrew University Medical Center, Kiryat Hadassah,
Jerusalem, Israel
Purpose. Vitreoretinal lymphoma is the most common type of intraocular
lymphoma, and in most cases accompanies CNS lymphoma. Radiation
therapy and systemic chemotherapy are the traditional methods
of treating vitreoretinal lymphoma, but in recent years intravitreal
chemotherapy has been used successfully in several centers.
Methods. Since 1997 we have treated all our patients with vitreoretinal
lymphoma by intravitreal injections of 400 µg/0.1 ml methotrexate
alone. Our protocol includes twice-weekly injections for 4 weeks, a
weekly injection for 8 weeks, and a monthly injection for 9 months, for
a total of 25 injections.
Results. Over the last 14 years we have treated 80 eyes of 45 patients;
in three-quarters of them the disease was bilateral. In 41 patients the
lymphoma was of the B-cell type, and in 4 the T-cell type. In one-third of
the patients the vitreoretinal lymphoma preceded the CNS lymphoma,
and in the rest the brain lymphoma was diagnosed earlier. All our
patients responded to the treatment with total disappearance of the
lymphoma cells from the vitreous and the infiltrates from the retina after
3 – 16 injections. In all but one patient no recurrence was noticed. In
one patient recurrence was diagnosed in one of the two affected eyes,
and was treated again by our protocol, with complete response. Irritated
conjunctiva and corneal epitheliopathy were the most common side
effects. Four patients, two of them diabetics, developed neovascular
glaucoma that recently was treated using intravitreal injections of
Avastin.
Conclusions. Intravitreal injection of methotrexate is a very successful
method of treating vitreoretinal lymphoma, with almost no recurrence
and reasonable ocular side effects.
Financial disclosure. None.
EYELID, CONJUNTIVA & ORBIT
Abstracts
74
2329 OT12
COMBINATION INTRAVITREAL RITUXIMAB AND
METHOTREXATE FOR PRIMARY VITREORETINAL
LYMPHOMA
David J. Wilson, MD and Justine Smith, MD, PhD (wilsonda@ohsu.edu)
Casey Eye Institute, Oregon Health and Science University
Purpose. To determine the response rate and ocular effects of monthly
intravitreal injection of a combination of methotrexate and rituximab in
the treatment of primary vitreoretinal lymphoma.
Methods. This is a retrospective chart review of a consecutive case series
from one institution of patients with primary vitreoretinal lymphoma that
were treated with combined methotrexate (.4mg/.1ml.) and rituximab (1
mg/.1ml) by intravitreal injection. Injections were given on a monthly
basis. Inclusion criteria consisted of biopsy proven vitreous or CNS.
CD20 positive lymphoma.
Results. Ten patients met the inclusion criteria. Five patients achieved
a complete remission (defined as complete absence of vitreous cellsor
sub RPE infiltrates). Five patients achieved partial remission (defined
as markedly reduced but still detectable vitreous cells). Median follow
up is 11 months. No ocular side effects directly attributable to the
intravitreous agents were detected.
Conclusions. Intravitreal injection of a combination of Rituximab and
Methotrexate apppears to be a safe method for inducing partial or
complete remission of a high percentage of patients with primary
vitreoretinal lymphoma.
Financial disclosure. None

2128 RF1
RETINOBLASTOMA
David H. Abramson, MD (abramsod@mskcc.org)
Memorial Sloan-Kettering Cancer Center, New York, N.Y. USA
Financial disclosure. None
1941 RF2
EXTRA-OCULAR RETINOBLASTOMA WITH INVASION
OF THE OPTIC CHIASMA: CAN BE SAVED?
Camila H. Hashimoto1, Carla R.D. Macedo1, Luiz F. Teixeira1,2, Virginia
L. Torres1,2, Juliana dos Santos Soares1, Maria T. Seixas1,3, Clelia M.
Erwenne1,2
(camilahashimoto@yahoo.com.br)
1. Pediatric Oncology Institute/GRAACC/Unifesp
2. Ophtalmology Department/ Unifesp
3. Pathology Department /Unifesp
Purpose. To describe an unusual case of a six year old boy with atypical extra-ocular
retinoblastoma with invasion of the optic chiasma
Methods. A case report of an atypical extra-ocular retinoblastoma.
Results. A 6 year old boy was referred to our service with a 7 months history of
gradual redness, photophobia, tearing, and pain in the right eye, ptosis and impaired
eye movements for the last 2 months. He had no family history of retinoblastoma.
At physical exam he had axial proptosis of the right eye. It was impossible to perform
fundoscopy in the right eye, and the fundoscopy of the left eye was normal.
Brain and orbit CT showed no calcification. The brain and orbit MRI showed an
enlargement of the right optic nerve until optic chiasma with a small tumor in the
right eye, with invasion of the subarachnoid space.
The differential diagnosis were atypical retinoblastoma, optic nerve tumor with
intraocular invasion, optic glioma, optic disc tumor with optic nerve invasion and
optic disc medulloepithelioma.
Systemic workup was normal.
We perform anterior approach enucleation and intraorbital segment of optic nerve
resection. The diagnosis was poorly differentiated retinoblastoma with extensive
choroidal invasion, invasion of the ciliary body and iris and invasion of tumor cells
into the optic nerve posterior to the lamina cribosa.
The treatment was chemotherapy and external beam radiotherapy of the orbit with
45Gy and CNS and spine with 24Gy.
He is alive without disease in a 8 years of follow-up.
Conclusions. Multimodality treatment with surgery, chemotherapy and radiotherapy
were primordial for the good result.
Financial disclosure. None
54 RF3
SEVEN-YEARS-OLD BOY WITH PERSISTENT PAINFUL
RED RYE AND PARS PLANA LESION
P. Chévez-Barrios, I. Hernandez, M. Chintagumpala, R. Hurwitz R, S.
Wittenberg, D. Moreno E. Passey, J. Edmond, C. Herzog, P. Zage, D. Gombos
(pchevez-barrios@tmhs.org)
Retinoblastoma Center of Houston; The Methodist Hospital Research
Institute; Baylor College of Medicine; Houston Eye Associates;
University of Texas, MD Anderson Cancer Center, Houston, TX, USA
Purpose. The case is presented as an unknown to highlight the
differential diagnosis and the importance of a team approach in the
Rapid Fire Cases
Abstracts
75
diagnosis of this masquerading presentation.
Methods. Seven-year-old boy presented with red eye and pain that
exquisitely responded to topical steroids for about a year. He also had
smoldering peripheral retina/pars plana lesion that in the last 2 months
increased in size. The most recent findings were anterior chamber debris
and increased intraocular pressure.
Results. The patient underwent anterior chamber tap with intraoperative
cytologic interpretation that will be presented.
Conclusions. The multidisciplinary approach, cytopathologic diagnosis
and management of the patient will be discussed.
Financial disclosure. None
1830 RF4
MALIGNANT TRANSFORMATION OF RETINOCYTOMA
Eduardo F. Marback, MD, Epaminondas de Souza Mendes Jr, MD,
Roberto L. Marback, MD (eduardomarback@uol.com.br)
Federal University of Bahia, Brazil
Purpose. To present a case of retinocytoma with malignant
transformation
Methods. Case report
Results. 37 year old man, was seen for an ocular oncology evaluation
because of having pain around the right eye for the past 15 days and
a progressive decrease in visual acuity for the past 3 years. He had
been evaluated in our service in 2001, after having two sons diagnosed
with bilateral retinoblastoma. At that time he had an ophthalmoscopic
appearance suggestive of bilateral retinocytoma and was adviced to
have regular ophthalmologic follow up at least once a year. Now he
returns with fundus pictures taken in 2008, showing a enlargement of
the tumors in the right eye. His evaluation showed a blind painful right
eye, with corneal edema, a mydriatic pupil with ectropion uvea and hazy
media. Ultrasonography and CT scan revealed an enlarged intraocular
mass, retinal detachment and an enlarged optic nerve. The right eye was
enucleated.
Conclusions. Retinocytoma patients should have periodical ophthalmic
evaluation. The authors pose a question about possible benefits of
prophylactic treatment is such cases.
Financial disclosure. None
1937 RF5
SUDDEN VITREOUS HAEMORRHAGE
Tero Kivelä (tero.kivela@helsinki.fi)
Department of Ophthalmology, Helsinki University Central Hospital,
Helsinki, Finland
Purpose. A medium-aged woman presented with sudden vitreous
haemorrhage, a choroidal mass in the papillomacular area and retinal
vascular malformations. The case will be presented as an unknown.
Financial disclosure. None
2044 RF6
SPONTANEOUS ISCHEMIA AND PIGMENT RELEASE
IN AN ADVANCED CASE OF RETINOBLASTOMA
D. Gombos MD FACS, P. Chevez-Barrios MD, P. Zage MD, C. Herzog MD,
M. Chintagumpala MD, R. Hurwitz (dgombos@mdanderson.org)

The Retinoblastoma Center of Houston; MD Anderson Cancer Center;
Texas Children’s Cancer Center; The Methodist Hospital Research
Institute; Baylor College of Medicine, Houston, Texas, USA
Purpose. Massive ischemia and necrosis can occur in advanced cases
of retinoblastoma. Few reports have documented clinical findings just
prior to and just after the ischemic event.
Methods. A single case report.
Results. The case will demonstrate the clinical findings just prior
to and after a massive ischemic event in a patient with bilateral
retinoblastoma. Photographic, radiographic and histologic correlation
will be presented.
Conclusions. This case demonstrates the atypical findings that can
occur in advanced eyes with retinoblastoma that develop a massive
ischemic event.
Financial disclosure. Dr. Gombos is a member of the Children’s Oncology Group and has
received reimbursement for travel. He is a paid consultant for IMS Health.
121 RF7
SUPRA-SELECTIVE CATHETERIZATION OF THE OPH-
THALMIC ARTERY FOR INTRA-ARTERIAL CHEMO-
THERAPY INJECTION IN RETINOBLASTOMA
Vicktoria (Vicky)Vishnevskia-Dai1, Iris Moroz1 , Joseph Moisseiev,
Mordechai Rosner1, Mati Bakon2
(vivida65@gmail.com)
1.Goldschleger Eye Institute, Sheba Medical center, Sackler School of
medicine, Tel-Aviv University Israel.
2. Invasive neuro-radiology center, Sheba Medical center, Sackler
School of medicine, Tel-Aviv University Israel.
Purpose. A 3-year old girl with unilateral group D retinoblastoma was
treated with Supra-selective catheterization of the ophthalmic artery
and intraarterial melphalan injections.
Financial disclosure. None
1957 RF8
PARANEOPLASTIC VITELLIFORM RETINOPATHY
M. Turell1, G. Adamus2, Y. Wang3, C.C. Chan3, A. Singh1
(turellm2@ccf.org)
1. Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland
Clinic, Cleveland, OH
2. Casey Eye Institute, Oregon Health & Science University, Portland, OR
3. Laboratory of Immunology, National Eye Institute, National Institutes
of Health, Bethesda, MD
Purpose. An 80-year old man was referred for evaluation of choroidal
metastases. The case will be presented as an unknown for opinions
from the audience.
Methods. Fundus photos, Fluorescein angiography, and OCT are
presented.
Results. Ophthalmic imaging exludes the possibility of choroidal
metastases. Serum autoantibodies additionally confirm the
paraneoplastic nature of the disease.
Conclusions. The clinical picture is consistent with the rare disease,
paraneoplastic vitelliform retinopathy.
Financial disclosure. None
Rapid Fire Cases
Abstracts
76
214 RF9
CILIARY BODY TUMOR IN A CHILD
Adriana C. Fandiño MD, Raslawsky E. Cristina MD, Chantada Guillermo
MD (acfandino@gmail.com)
Ophthalmology Department; Radiotherapy Department;
Hematooncology Department, Hospital de Pediatria J.P.Garrahan,
Buenos Aires, Argentina
Purpose. When a 10-year-old boy presented with rapid visual loss
because of progressive astigmatism, a ciliary body mass with rapid
progression was found.
Methods. It was assumed to be a medulloepithelioma and treated with
brachytherapy.
Results. The patient had an excellent course with more than eight year
follow-up.
Conclusions. The case will be presented for opinions from the audience
Financial disclosure. None
418 RF10
RETINAL DETACHMENT AFTER FNAB
Arturo Irarrazaval, Pablo Cazón (artuira@consultoresoftalmologicos.com)
Consultores Oftalmológicos
Purpose. To show a case where after the FNAB a retinal detachment that
did not resolve occurred.
Methods. Retrospective study of the patient chart
Results. The patient was first treated with pneumatic retinopexy, and
after that with vitrectomy, which resolved the detachment.
Conclusions. FNAB, although rarely, can produce a retinal detachment,
that can be succesfully treated with retina surgery.
Financial disclosure. None
2047 RF11
DIFFUSE MELANOCYTIC NEOPLASM IN A 9 YEAR-OLD
FEMALE WITH OCULODERMAL MELANOCYTOSIS
A.C. Schefler, A.M. Schimel, A. Abbey, E. Hodapp, S.R. Dubovy
(aschefler@med.miami.edu)
Bascom Palmer Eye Institute, University of Miami Miller School of
Medicine, Miami, Florida, U.S.A.
Purpose. This is an unusual case of a diffuse melanocytic malignancy
involving 360 degrees of the ciliary body and the entire choroid in a 9
year-old girl with oculodermal melanocytosis. The patient presented with
melanocytomalytic glaucoma and ultimately underwent enucleation.
Extensive clinical photographs and histopathology will be shown.
Financial disclosure. None
2251 RF12
A CASE OF ADENOMA OF THE NON-PIGMENTED
CILIARY EPITHELIUM CAUSING NEOVASCULAR
GLAUCOMA
Vasilios P. Papastefanou, Victoria ML Cohen (bazmed@hotmail.com)

Ocular Oncology Service, St Bartholomew`s Hospital and Moorfields Eye
Hospital, London, UK
Purpose. A 67-year old Caucasian man presented with rapidly reducing
vision in the right eye. Reduced vision was attributed to cataract and he
underwent routine cataract surgery. However, intraoperatively a solid
mass was noted. The patient was referred to the Ocular Oncology Service.
Methods. Transillumination did not reveal a clear shadow and B scan
ultrasound revealed a ciliary body mass with internal blood flow and
medium internal reflectivity. An incisional biopsy was performed. The
histology results are discussed. A diagnosis of ciliary body adenoma of
the non-pigmented epithelium (NPCE adenoma) was made.
Results. One year later, patient developed hyperemia with blurred vision
in the affected eye. He had marked episcleral injection, an oedematous
cornea, marked rubeosis and angle closure. The intraocular pressure
was 42 mmHg. The ciliary body adenoma remained unchanged .
The tumour was treated with plaque radiotherapy (Ru-106) in order to
restrict the neovascular response. Treatment was successful in reducing
the tumour size on B-scan ultrasonography. Intracameral injections of
bevacizumab where required pre- and postoperatively to control the rubeosis.
Conclusions. To our knowledge, this is the first report of neovascular
glaucoma secondary to a ciliary body adenoma.
Financial disclosure. None
1934 RF13
CHOROIDAL NEVUS...CHOROIDAL NEVUS?
Miguel A. Materin, Ruth Halaban, Antonella Bacchiocchi (miguel.materin@yale.edu)
Yale School of Medicine
Purpose. 10 years follow up on a typical choroidal nevus
Conclusions. To be discussed at the meeting.
Financial disclosure. None
2312 RF14
MUSHROOM SHAPED TUMOR. MELANOMA OR NOT?
Jerry Shields, MD (jerryshields@comcast.net)
Wills Eye Institute
Purpose. To present a mushroom shaped tumor
Methods. Review of case
Results. Diagnosis and management
Conclusions. A mushroom shaped tumor could be melanoma or
something else.
Financial disclosure. None
104 RF15
AMELANOTIC INTRAOCULAR LESION IN A 26 YEAR-
OLD FEMALE PATIENT
Luiz F. Teixeira1,2, Carla R. D. Macedo2, Carlos A.G. Filho1, Camila H.
Hashimoto2, Marcia Lowen3, Maria C. Martins1 (luizfteixeira@hotmail.com)
1. Department of Ophthalmology- Federal University of Sao Paulo-
UNIFESP
2. Pediatric Oncology Institute/GRAACC/UNIFESP
3. Department of Pathology- Federal University of Sao Paulo
Rapid Fire Cases
Abstracts
77
Purpose. A 26 year-old female patient presented with an unusual
intraocular lesion. It will be presented as a mysterious case for
discussion.
Financial disclosure. None
2328 RF16
PIGMENTED ANTERIOR SEGMENT TUMOR IN A YOUNG
MAN
Carolina M. Gentile, Camila Challiol, Atilio Lombardi, J. Oscar Croxatto
(carolina.gentile@gmail.com)
Hospital Italiano de Buenos Aires, Argentina
Purpose. The aim of the presentation is to discuss the diagnosis and
management of an anterior melanocytic segment tumor in a young adult.
Methods. A 38 years-old male high mountain climber presented with a
growing anterior segment pigmented mass. Diagnosis and management
will be discussed with the audience. It will be presented as an unknown
case for opinions.
Results. Fine needle aspiration biopsy was performed and the diagnosis
was melanocytoma.
Conclusions. Anterior uveal melanocytomas are uncommon benign
melanocytic tumors. Despite the benign nature of iris melanocytoma,
growth was observed and histologic confirmation was necessary to
establish a diagnosis and ruled out malignancy.
Financial disclosure. None
2254 RF17
AMELANOTIC CHOROIDAL MASS EXTENDING TO THE
FOVEA
Zelia M. Correa, MD, PhD, James J. Augsburger, MD (correazm@uc.edu)
Dept of Ophthalmology, University of Cincinnati, Cincinnati, OH, USA
Purpose. Case presentation
Methods. A 66-year-old asymptomatic Caucasian female was found to
have a choroidal mass measuring 14 X 12 mm in base and 2.5 mm in
thickness, extending to the fovea +/- SRF on routine eye exam.
Results. The importance of accurate diagnosis will be discussed.
Conclusions. Clinical diagnosis impacts management decision.
Financial disclosure. None
29 RF18
PIGMENTED CHOROIDAL TUMOR
Jacob Pe’er, MD, Shahar Frenkel, MD, PhD
(peer@hadassah.org.il)
Specialized Ocular Oncology Service, Department of Ophthalmology,
Hadassah - Hebrew University Medical Center, Jerusalem, Israel
Purpose. An 84-year-old woman presented with elevated (6.9 mm)
pigmented lesion with retinal and choroidal hemorrhage around it.
The lesion was diagnosed as choroidal melanoma and treated using
brachytherapy, with good response.
One year later regrowth was noticed, and the eye was enucleated.
There was a surprise in the histopathologic examination.
Financial disclosure. None

2028 RF19
RAPID REGRESSION OF POST-BRACHYTHERAPY
CHOROIDAL MELANOMA IN RESPONSE TO
INTRAVITREAL AVASTIN
Peter Hovland MD PhD (peterhovland@gmail.com)
Colorado Retina Associates, Denver CO
Purpose. To describe the rapid decrease in tumor volume seen in a
patient treated with intravitreal avastin following brachytherapy.
Methods. A 65 year old woman with anteriorly located choroidal
melanoma of 10 mm thickness was treated with I-125 brachytherapy.
After 6 months, persistent symptomatic sub-retinal fluid was treated
with intravitreal avastin. Tumor thickness was monitored by b-scan
ultrasound.
Results. FNAB analysis of the tumor confirmed it to be a melanoma with
a GEP Class 1. Tumor volume gradually reduced from a pre-treatment
10.3 thickness to 8.0 mm in the first 6 months. Following 2 injections of
intravitreal avastin, the tumor thickness decreased to less than 1 mm.
Conclusions. Intravitreal avastin injections were associated with a rapid
decrease in tumor volume in a patient with a large choroidal melanoma
post-brachytherapy.
Financial disclosure. None
1845 RF20
LIGHT AND ELECTRON MICROSCOPY OF TWO CILIARY
BODY TUMORS
L. Schoenfield, J. McMahon, A. Singh (schoenl@ccf.org)
Cleveland Clinic
Purpose. To discuss the differential diagnosis of two ciliary body
tumors.
Methods. Two cases of ciliary body tumors were evaluated with routine
H&E stains, immunoperoxidase stains, and electron microscopy.
Results. To be discussed at rapid fire section.
Conclusions. Two tumors from the ciliary body of a 70 year old male and
a 77 year old female will be presented with light microscopy (including
immunoperoxidase stains) and electron microscopy. Discussion and opinions
from the audience will be welcomed as to the nature of these lesions.
Financial disclosure. None
513 RF21
RETINAL DETACHMENT AFTER TTT FOR ACTIVE
CHOROIDAL MELANOMA
Prithvi Mruthyunjaya, MD (mruth001@mc.duke.edu)
Ocular Oncology and Vitreoretinal Surgery Services, Duke Eye Center,
Durham, NC
Purpose. 54 yo male with choroidal melanoma and exudative retinal
detachment has undergone plaque brachytherapy with continued
growth. Transpupillary thermotherapy is applied and a subsequent
rhegmatogenous retinal detachment develops. What is the ideal timing
of RD repair in the setting of an active melanoma?
Methods. Case report
Results. Audience participation requested
Conclusions. What is the ideal timing of RD repair in the setting of an
Rapid Fire Cases
Abstracts
78
active melanoma?
Financial disclosure. None
55 RF22
TREATMENT OF DIFFUSE IRIS MELANOMA
B. Damato (bertil.damato@gmail.com)
Liverpool, UK.
Purpose. Succesfull treatment of diffuse iris melanoma after proton
beam radiotherapy of the entire anterior segment
Financial disclosure. None
62 RF23
LATE RECURRENCE OF A PRESUMED IRIS MELANO-
MA
John McWhae, Ryan Steinke (jmcwhae@gmail.com)
Purpose. A 65-year old woman presented with a spontaneous hyphema
OD. She has a history of iris melanoma treated with iridectomy 35 years
prior. She was found to have 5 small amelanotic angle lesions. The
patient refused any interventions. A second hyphema occurred 3 years
later. The patient has now been followed for 5 years without any growth
of the lesions. Possible explanations for this unusual tumor behaviour
will be discussed.
Financial disclosure. None
45 RF24
HOLY TUMOR
Carol Shields, M.D. (carol.shields@shieldsoncology.com)
Wills Eye Institute
Purpose. An interesting case.
Methods. Patient with uveal melanoma.
Results. Halo nevi developed.
Conclusions. An interesting cutaneous phenomenon developed
following treatment of uveal melanoma in a young woman.
Financial disclosure. None
1848 RF25
RESECTION OF AN IRIS TUMOR
Arun D Singh, Laura Snyder, Linda Brodell, Mary B. Turell (singha@ccf.org)
Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland
Clinic, Cleveland, OH
Purpose. A 71-year old Caucasian female was noted to have a small, light
brown mass extending into the angle without iris deformity. On follow
up examination sixteen months later, a new finding of iris distortion was
observed.
Financial disclosure. None
410 RF26
MELANOMA OR MELANOCYTOMA?

Arturo Irarrazaval, Pablo Cazon, Oscar Croxatto
(artuira@consultoresoftalmologicos.com)
Consultores Oftalmologicos
Purpose. To show an unusual evolution of a plaque treated tumor of the
posterior pole.
Methods. A patient treated with plaque for a mushroom shaped choroidal
melanoma, with characteristic ecography, that had quick growth after
some years of stability, even after TTT adjuvant treatment.
Results. The pathology of enucleated eye shows that the tumor was a
melanocytoma
Conclusions. A choroidal melanocytoma may have an unusual form of
presentation and evolution.
Financial disclosure
None
1655 RF27
MALIGNANT TRANSFORMATION OF CELLULAR BLUE
NEVUS IN AN 8-YEAR-OLD GIRL
A P Moulin1, M Hamedani1, A Oberic1, M Mihm2, F Jakobiec3, V Prieto4, JN
Myers4, F Demont4, RJ Skoracki4, S Woodman4, B Eemaeli4. (alexandre.
moulin@fa2.ch)
1. Jules Gonin Eye Hospital, Lausanne University, Switzerland
2. Brigham and Women’s Hospital, Harvard Medical School, Boston
3. Massachussetts Eye and Ear Infirmary, Harvard Medical School,
Boston
4. MD Anderson Cancer Center, University of Texas, Houston
Purpose. To report a case of malignant transformation of blue nevus of
eyelid into melanoma of orbit in an 8-year-old girl.
Methods. Observational case report.
Results. An 8-year-old girl was referred to Jules Gonin Eye Hospital
for evaluation of an extensive blue nevus involving the right lower
eyelid, the inferior conjunctiva and the orbit with evidence of recent
growth into the orbit. Multiple palpebral and orbital biopsies were
performed. The deepest lesions were consistent with cellular blue
nevus with severe atypia. After 6 months observation, growth of the
orbital mass led to repeat biopsies which confirmed transformation
into invasive melanoma in the orbit. aCGH demonstrated a complete
gain of chromosome 6. There were no mutations of BRAF (exon 15),
GNAQ and GNA11 (exons 5). The patient was referred for evaluation
and management to the University of Texas M.D. Anderson Cancer
Center. The orbital tumor was found to involve the pterygopalatine
fossa and other skull base structures. A multidisciplinary
procedure including an orbital exenteration and skull base surgery
was performed followed 4 weeks later by proton radiotherapy.
Histopathology revealed atypical cellular blue nevus with foci of
melanoma in the posterior orbit.
Conclusions. Cellular blue nevi can rarely transform into malignant
melanoma and can involve deeper structures in the orbital apex and
skull base. To our knowledge, this may be the youngest patient to
date documented to have a malignant transformation of cellular
blue nevus into melanoma. The extent of involvement of atypical
blue nevus required a complex multidisciplinary surgical approach
followed by high-dose radiation therapy.
Financial disclosure. None
Rapid Fire Cases
Abstracts
79
1618 RF28
ORBITAL MELANOMA AND NAEVUS OF OTA
Victoria M.L. Cohen (victoria.lendrum@gmail.com)
Ocular Oncology Service, Moorfields and St Batholomew’s Hospital
London, UK.
Purpose. To report the management of an extensive orbital melanoma
in a patient with naevus of Ota
Methods. A 46-year old male with long standing pigmentation of the
eye and eyelid presented with progressive proptosis. Orbital biopsy
confirmed the presence of melanoma. Despite adjuvant radiotherapy,
the lesion continued to enlarge. The subsequent radical surgical
approach is described
Results. Melanoma was found to be infiltrating the orbital bone,
maxilla, nasopharynx and dura mata.
Conclusions. Orbital and cerebral melanoma is very rare but the most
lethal association of naevus of Ota.
Financial disclosure. None
558 RF29
MANAGEMENT OF A RARE ADNEXAL TUMOR
Sonul Mehta, MD1, John Harvey, MD, F.R.C.S.2 (sonulmehta@gmail.
com)
1. Division of Oculoplastics and Orbital Surgery, Department of
Ophthalmology and Visual Sciences, University of Toronto
2. Division of Oculoplastics and Orbital Surgery, Department of
Ophthalmology and Visual Sciences, McMaster University
Purpose. A 28 year old male who sustained a right upper brow and
eyelid laceration after an injury that presented with a non-healing
wound 3 months later. He was found to have a rare adnexal tumor.
He will be presented to the audience as an unknown for opinions on
management.
Methods. Case Study
Results. He was found to have a rare adnexal tumor that has failed
multiple reconstructions and found to have recurrence of tumor.
Conclusions. He will be presented to the audience as an unknown for
opinions on management.
Financial disclosure. None
19 RF30
AMELANOTIC CONJUNCTIVAL TUMOR - MALIGNANT OR
BENIGN?
Priscilla Ballalai, Maria C. Martins, Marcia Lowen (pbbordon@terra.com.br)
Federal University of Sao Paulo
Purpose. 82 yo female with a rapid growing conjunctival tumor
Financial disclosure. None
1809 RF31
65-YEAR OLD WOMAN WITH DIFFUSE CONJUNCTIVAL
NEOPLASM
J. William Harbour, MD (harbour@vision.wustl.edu)

Washington University, St. Louis, Missouri
Purpose. This case will be presented as an unknown for discussion of
diagnosis and management.
Methods. Case presentation.
Results. This is a 65 year old Caucasian woman who presents with
a diffuse conjunctival neoplasm involving her right eye. Three years
prior to presentation, she noticed a loss of vision in her superior
visual field in the right eye but did not seek medical attention
because she was uninsured. Over the past 18 months she has noticed
that the white part of the right eye has gradually become discolored.
On examination, she had no pain. There was no light perception and
the intraocular pressure was 56 in the right eye. Anterior segment
examination revealed diffuse thickening and hypervascularity of
the conjunctiva, which had a dark gelatinous appearance with an
irregular, roughened surface. A view of the posterior segment was
precluded by a dense cataract. Ultrasonography of the right eye
revealed a total retinal detachment with diffuse thickening of the
choroid and retina but no discrete mass. An intraconal orbital mass
was also noted, which was discontinuous from the globe. The left eye
examination was unremarkable.
Conclusions. Final diagnosis and management will be discussed.
Financial disclosure. None
23 RF32
DIFFUSE CONJUNCTIVAL MALT LYMPHOMA
Shahar Frenkel, MD, PhD, Jacob Pe’er, MD
(shahar.frenkel@gmail.com)
Specialized Ocular Oncology Service, Department of Ophthalmology
Hadassah - Hebrew University Medical Center, Jerusalem, Israel
Purpose. A 40-year-old man was diagnosed as suffering from diffuse
(almost 360deg) conjunctival lymphoma of the right eye. There
was no evidence of other sites of lymphoma. He was treated via six
courses of Rituximab, with complete response. No recurrence was
seen in follow-up of four years.
Any other ideas for treatment in such a case?
Financial disclosure. None
1510 RF33
PAEDIATRIC TUMOUR WITH SCLERAL INVASION
Maria Tsimpida, Amit Arora, Victoria Cohen, Mary Lendrum, John
Hungerford(tsimpidam@yahoo.co.uk)
The Ocular Oncology Service St. Bartholomew’s and Moorfield’s Eye Hospitals
Purpose. A 15 year-old boy presented with a reddish-blue domed shaped
intrascleral lesion in the upper temporal quadrant of the right eye. There
was associated scleral thinning. A corresponding irregularly shaped, pale,
choroidal mass was noted. Cells were present in the vitreous.
Methods. B-scan ultrasonography showed an anterior choroidal mass with
internal blood flow. This was further detailed with a UBM. There was no
evidence of ocular inflammatory or systemic collagen vascular disorder.
Results. The lesion was observed, as diagnostic biopsy would be hasardous
in view of the overlying scleral thinning.A year later, the scleral nodule
Rapid Fire Cases
Abstracts
80
increased in size and the intraocular lesion had extended to involve the ciliary
body. Interestingly, a cyst was found adjacent to the ciliary body mass.
Conclusions. There was concern that this mass was a medulloepithelioma
that was invading the sclera and the patient was offered enucleation. The
surprising histopathological results are discussed.
Financial disclosure. None
1227 RF34
STEREOTACTIC-GUIDED TRANSCRANIAL CRYOEXTRACTION
OF CAVERNOUS HEMANGIOMA IN THE ORBITAL APEX
Mordechai Rosner1, Sagi Harnof2, Vicktoria (Vicky) Vishnevskia-Dai1,
Nachum Rosen1 (mrosner@post.tau.ac.il)
1. Goldschleger Eye Institute, Sheba Medical center, Sackler School of
medicine, Tel-Aviv University Israel.
2. Department of Neurosergery, Sheba Medical center, Sackler School
of medicine, Tel-Aviv University Israel.
Purpose. A 55-year old man with cavernous hemangioma at the apex of his
right eye underwent stereotactic guided transcranial cryoextraction with
favorable results.
Financial disclosure. None
1352 RF35
MYSTERIOUS ORBITAL CASE
Patrick De Potter (patrick.depotter@uclouvain.be)
Ocular Oncology Unit, Cliniques Universitaires St-Luc, Brussels, Belgium
Purpose. Presentation of a mysterious orbital lesion
Financial disclosure. None
1802 RF36
ORBITAL INVOLVEMENT FROM AN UNUSUAL CONDITION
Manquez Maria (m_manquez@yahoo.com)
Clinica Oftalmologica Pasteur, Santiago Chile
Purpose. 58-year old Hispanic male with orbital mass and unusual
liver condition
Financial disclosure. None
112 RF37
REPONSE OF METASTATIC PANCREATIC
ADENOCARCINOMA TO CHEMOTHERAPY
Scott Oliver, MD scott.oliver@ucdenver.edu)
Deparment of Ophthalmology, University of Colorado School of Medicine
Purpose. A case of pancreatic adenocarcinoma presenting with
circumpapillary choroidal metastasis will be presented. Management
options will be discussed, and dramatic response to gemcitabine and
ipilimumab will be shown.
Financial disclosure. None
127 RF38

A CASE OF TEARING AND SWELLING OF THE EYE AFTER
RESOLUTION OF ORBITAL CELLULITIS
Daniel Moreno-Páramo1, David M. Brown2, Robert W. Wong3, Dan S.
Gombos1,2,4,5, Milton Boniuk4, Patricia Chévez-Barrios1,2,4,5
(danomp@hotmail.com)
1. The University of Texas, MD Anderson Cancer Center, Houston, Texas
2. The Methodist Hospital, Weill Cornell Medical College of Cornell
University, Houston, Texas
3. Austin Retina Associates, Austin, Texas
4. Baylor College of Medicine, Houston, Texas
5. Retinoblastoma Center at Houston, Houston, Texas
Purpose. To present as “mystery case” the findings of a 78-yearold
woman with tearing and swelling of the left eye for discussion of
differential diagnosis, pathology diagnosis and treatment.
Methods. A 78-year old Caucasian woman presented with tearing and
swelling of the left eye and history of orbital cellulitis of the same side.
Imaging studies and fundoscopy showed a flat white lesion in the choroid
and a larger lesion in the orbit.
Results. Imaging, pathology and laboratory findings will be presented.
Conclusions. Clinical and histopathologic differential diagnosis and
management will be discussed.
Financial disclosure. None
124 RF39
MYSTERY CASE
Masood Naseripour MD (masoodnp@yahoo.com)
Eye Research Center, Rassoul Akram Hospital, Tehran University of Medical
Sciences
Purpose. To report an interesting case
Methods. A 5-month-old baby referred with an unusual black retinal
mass. It will be presented as an unknown case for opinions from the
audience.
Conclusions. Will be presented
Financial disclosure. None
158 RF40
A PATIENT WITH MULTIPLE CANCER HISTORY
Helen K. Li, MD (hlimed@mac.com)
1. The Methodist Hospital / Weil Cornell Medical College, Houston, TX
2. The University of Texas Health Science Houston, Houston, TX
3. Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA.
Purpose. To be presented as an unknown case for opinions from the
audience.
Financial disclosure. None
110 RF41
MULTIPLE OCULAR VASCULAR ANOMALIES IN A CHILD
Mandeep S. Sagoo1,2,4 MB, PhD, MRCOphth, FRCS (Ed), Wisam J.
Muen1,2 MRCS.Ed(Surg), FRCOphth, Clare Roberts3 MA, FRCOphth, M.
Ashwin Reddy1,2 MD, FRCOphth (mandeep.sagoo@moorfields.nhs.uk)
1. Retinoblastoma Unit, Barts & the London NHS Trust, Royal London
Rapid Fire Cases
Abstracts
81
Hospital, Whitechapel Road, London, UK
2. Moorfields Eye Hospital, City Road, London, UK
3. Moorfields Dubai Eye Hospital, Dubai, UAE
4. UCL Institute of Ophthalmology, Bath Street, London, UK
Purpose. A 2-year-old white female with ocular vascular anomalies is
presented for discussion.
Financial disclosure. None
1938 RF42
A MYSTERY CASE
M. Ashwin Reddy MD FRCOph1,2, Sharola Dharmaraj PhD FRCS1,2,
Mandeep Sagoo PhD FRCS FRCOph1,2,3, Himanshu Patel MS FRCS1,2,
Phil Luthert PhD FRCP FRCPath2,3
(mashwinreddy@hotmail.com)
1. Barts and The London
2. Moorfields Eye Hospital
3. Institute of Ophthalmology
Purpose. A 2-year old boy presented with a squint and an unusual red
reflex. Fundus examination demonstrated a calcified mass with a fibrotic
front of blood vessels. To be presented as a mystery case.
Financial disclosure. None
1834 RF43
MYSTERY CASE
Edoardo Midena1,2, Raffaele Parrozzani2 (edoardo.midena@unipd.it)
1 Department of Ophthalmology, University of Padova, Padova, Italy.
2 GB Bietti Eye Foundation, IRCCS, Roma, Italy.
Purpose. A 46-year-old woman affected by a left choroidal successfully-treated
mass underwent massive, perilesional, purely serous retinal detachment.
Financial disclosure. None
1255 RF44
MYSTERY CASE
Michael E. Giblin (emgiblin@ozemail.com.au)
Sydney Eye Hospital, Sydney, Australia
Purpose. To demonstrate an unusual presentation of a rare fundal tumour.
Methods. A 29 year old white female patient presented with a
pigmented fundal mass, considered suitable for Transpupillary Laser
Thermotherapy (TTT). Immediately prior to the TTT some three weeks
later, the mass had significantly altered.
Conclusions. For audience to deduce
Financial disclosure. None
1309 RF 45
METASTATIC CHOROIDAL PARAGANGLIOMA
Ann Schalenbourg1, Alexandre Moulin1, Louis Guillou2 and Leonidas
Zografos1 (ann.schalenbourg@fa2.ch)
1. Jules-Gonin Eye Hospital, University of Lausanne, Switzerland
2. University Institute of Pathology and University Hospital, University
of Lausanne, Switzerland

Purpose. To describe a patient with metastatic choroidal paraganglioma,
locally controlled with radiotherapy.
Methods. Interventional clinico-pathological case report. Systematic
search of the literature.
Results. A 50-year-old man presented a non-pigmented atypical
choroidal mass with secondary retinal detachment in the left eye.
Following incisional biopsy, the diagnosis of paraganglioma was
established. Metastatic work-up revealed vertebral, mediastinal and
pulmonary metastases of a non secretory, malignant paraganglioma
without tracer uptake. The primary tumor was not identified. The ocular
tumor regressed after stereotaxic radiotherapy. Two years later, the
patient developed recurrent lesions in the contralateral eye, which were
also irradiated.
Conclusions. Malignant paraganglioma can metastasize in the choroid
and should be included in the differential diagnosis of a non pigmented
choroidal mass. Stereotaxic radiation therapy is an effective treatment
modality. To our knowledge, this is the first report of a patient with
choroidal paraganglioma.
Financial disclosure. None
Rapid Fire Cases
Abstracts
82
67 RF46
MYSTERY CASE
Sara Lally (saralally1@yahoo.com)
Wills Eye Hospital, Philadelphia
68 RF47
MYSTERY CASE
Hakan Demirci (hdemirc1@hfhs.org

1849 ECp101
FIVE CASES OF CARCINOMA METASTASIC TO THE
EYELIDS
Hiroya Kashiwagi1, Hirohisa Katagiri2, Takuya Takagi2, Hideki Murata2,
Mitsuru Takahashi2, Toshiaki Takahashi3 , Masato Matsuzaki4
(h.kashiwagi@scchr.jp)
1. Ophthalmology, 2. Orthopedics, 3. Thoracic Oncology, and 4. Urology,
of Shizuoka Cancer Center
Purpose. We report 5 rare cases of carcinoma metastasis to the eyelid.
Methods. We reviewed the medical records of 5 patients with metastatic
eyelid carcinoma who consulted the Shizuoka Cancer Center from April
2007 to March 2011.
Results. Four of the patients were male and one was female (age range,
42–71 years; average age, 62.4 years). The primary tumors were a seminal
vesicle leiomyosarcoma, an occult primary neuroendocrine carcinoma, a
lung cancer, occult primary sarcoma, and a renal cell carcinoma. The site of
carcinoma was the upper eyelid in 3 cases and the lower in 2 cases. The renal
cell carcinoma was the only tumor that was discovered before the primary
lesion was found. The treatment given was complete removal in 1 case,
excisional biopsy in 1case, and incisional biopsy in 3 cases. Three patients
died of systemic metastatic disease, and the time to death from discovery of
the lesion was 2–3 months (average, 2.3 months). The leiomyosarcoma was
resistant to chemotherapy and irradiation. The occult primary sarcoma was
treated with macroscopic complete extraction. Therefore, chemotherapy
was administered, and it was found to be effective with no recurrence. The
renal cell carcinoma showed rapid growth even after the primary carcinoma
was removed completely; therefore, complete resection with a safety margin
of 5 mm was performed, and no recurrence has been reported to date.
Conclusions. We think that early extraction are necessary for sarcoma.
Oncologists should also understand that eyelid lesions may progress even
if the primary tumor was removed.
Financial disclosure. None
1516 ECp102
NATURAL KILLER/T-CELL LYMPHOMAS IN OCULAR
LESION
Hideki Tsuji, Megumi Kobayashi, Kengo Takeuchi, Kazuhiro Oshitari,
Keigo Shikishima (tsuji-tky@umin.ac.jp)
The Cancer Institute Hosptal
Purpose. The importance of malignant lymphomas (ML) in ocular
oncology is widely known. However, NK/T ML constitute a extremely
small fraction of ocular adnexal lymphomas (OAL). We show the cases
of NK/T in OAL.
Methods. Case reports of NK/T malignant lymphomas (ML) of medial
rectus muscle (68y.o.M) and of eyelid (60 y.o. F).
Results. Both cases had aggressive clinical coarse even with radiation
and chemotherapy, especially the medial rectus muscle case recurred
and showed poor prognosis.
Conclusions. NK/T ML in OAL have different clinical course compared with
MALT lymphoma. These cases suggest us about clinical and pathological
varieties of OAL and each subtype ML requires each prompt therapeutic strategies.
Financial disclosure. None
2337 ECp103
CONJUNCTIVAL SQUAMOUS CELL CARCINOMA
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
83
ARISING IN IMMUNOSUPPRESSED PATIENTS (ORGAN
TRANSPLANT, HUMAN IMMUNODEFICIENCY VIRUS
INFECTION)
Carol L. Shields, M.D., Aparna Ramasubramanian, M.D., Phoebe L. Mellen,
B.S., Jerry A. Shields, M.D. (carol.shields@shieldsoncology.com)
Wills Eye Institute
Purpose. To describe the relationship between chronic systemic immune
suppression and conjunctival squamous cell carcinoma (SCC).
Methods. Surgical excision in all cases plus additional topical interferon
alpha 2B and/or mitomycin
Results. There were three groups of patients with chronic
immunosuppression and conjunctival SCC, including post organ
transplant (n=8), human immunodeficiency virus (HIV) (n=4), and
systemic lupus erythematosis (SLE) on long-term corticosteroids (n=1).
The transplanted organ was kidney (n=4), lung (n=2), liver (n=1),
and heart (n=1). The mean patient age at presentation for the organ
transplant group was 60 years and the mean interval from transplant
to conjunctival SCC was 8.2 years. Management included surgical
excision (n=8) plus additional topical interferon alpha 2B (n=3) and
mitomycin C (n=1). Three patients showed aggressive recurrence. In
the HIV group, the mean patient age at presentation was 54 years and
the mean interval from HIV diagnosis to conjunctival SCC was 5 years.
Management included surgical excision (n=5) plus additional topical
interferon alpha 2B (n=3) and mitomycin C (n=1). One patient showed
aggressive extensive recurrence. Of the 5 patients treated with excision
and prompt topical interferon alpha 2B, none showed recurrence or new
tumor.
Conclusions. Conjunctival SCC can occur in immune suppressed patients
and can be more aggressive and invasive, requiring enucleation or
exenteration. Surgical resection plus topical interferon alpha 2B might
reduce the risk for recurrence/new tumor.
Financial disclosure. None
1822 ECp104
SQUAMOUS NEOPLASIA OF THE OCULAR SURFACE
INVADING THE EYE AND ORBIT: A STUDY OF 30 CASES
Eduardo F. Marback, Ediney Vila Nova Silva, Roberto L. Marback
(eduardomarback@uol.com.br)
Federal University of Bahia - Brazil
Purpose. To evaluate cases of squamous neoplasia of the ocular surface
(SNOS) with orbital or intraocular invasion that required mutilating
surgery.
Methods. A review in the registry book from the Ophthalmic Pathology
Laboratory at Federal University of Bahia – Brazil from January 2000 to
December 2009 was conducted looking for entries with histopathologic
diagnosis of SNOS. Cases with intraocular or orbital invasion that
required mutilating surgery (eye enucleation or orbital exenteration)
were selected. Collected data included age, sex, preoperative visual
acuity, duration of symptoms and presence of previous surgery.
Results. From 213 cases of SNOS, 30 (14%) had conjunctival squamous
cell carcinoma that necessitated mutilating surgery. Thirteen (43%)
patients required eye enucleation and 17 (57%) required orbital
exenteration. In the enucleation group the mean age at diagnosis was
62,9 years-old (ranging from 42 to 95), 10 (77%) patients were male,
the mean duration of symptoms was 15,2 months (ranging from 3 to

36), 6 (46%) reported previous surgery. In the exenteration group the
mean age at diagnosis was 67,8 years-old (ranging from 31 to 88), 11
(64,7) patients were male, the mean duration of symptoms was of 28,5
months (ranging from 3 to 94), 6 (35%) reported previous surgery.
Conclusions. Mutilating surgery due to orbital or intraocular invasion
by SNOS is uncommon (14%). History of previous surgery was detected
in 40% of the cases. Duration of symptoms was 1.9 higher in cases
undergoing exenteration, a factor that may be considered important for
the occurrence of orbital invasion.
Financial disclosure. None
1150 ECp105
A PROPOSAL FOR A NEW TREATMENT OF ORBITAL
LOW GRADE MALIGNANT LYMPHOMA
Akihiro Kaneko (akikaneko@jcom.home.ne.jp)
Department of Ophthalmology, Teikyo University Hospital, Tokyo, Japan
Purpose. TO propose a new treatment for orbital low grade lymphoma to
avoid complications of radiotherapy
Methods. A selective ophthalmic arterial injection with 3-5mg of
melphalan (SOAI) is performed to eradicate orbital low grade malignant
lymphoma.
Results. The author has no chance to try this therapy until now.
Conclusions. Melphalan is an old alkylating agent which has
radiomimetic activity which means to work as radiotherapy.SOAI is
developed by our group for eye-preservation therapy of retinoblastoma.
It is performed more than 20 countries over the world, because very
slight complications are recognized.
Therefore good response is able to be expected for the treatment of
localized orbital lymphoma. in addition to that ,many complications of
radiotherapy must be avoidable.
Financial disclosure. None
59 ECp106
ORBITAL EXENTERATION RECONSTRUCTION WITH
RADIAL FOREARM AND ANTEROLATERAL THIGH FREE
FLAP: A MULTIDISCIPLINARY APPROACH
Sara E. Lally, M.D., Carol L. Shields, M.D., Joseph Curry, M.D., Ryan
Heffelfinger, M.D., David Cognetti, M.D., Howard Krein, M.D., Jerry A.
Shields, M.D. (saralally1@yahoo.com)
Wills Eye Institute, Philadelphia PA
Purpose.Orbital exenteration is a radical procedure to remove the eye
and orbital contents, usually for invasive malignancies. When possible,
eyelid skin and muscle are spared to aid in closure and healing. If tumor
infiltrates the eyelid, then this tissue must be removed. Reconstruction
of the socket with radial forearm and anterolateral thigh free flaps is
performed.
Methods. Review of technique and outcomes.
Results. Over the past 5 years, exenteration was performed in 44
cases for diagnoses of invasive eyelid squamous cell carcinoma (n=11),
invasive eyelid basal cell carcinoma (n=1), eyelid sebaceous carcinoma
(n=7), conjunctival melanoma (n=8), extraocular extension of uveal
melanoma (n=5), lacrimal gland adenoid cystic carcinoma (n=3),
primary orbital malignancies (n=7), and other extraocular extension of
intraocular tumors (n=2). In 33 cases, eyelid sparing exenteration and
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
84
reconstruction with primary closure was achieved. In 11 cases, eyelid
sacrificing exenteration was performed and reconstruction with radial
forearm (n=4) or anterolateral thigh (n=7) myocutaneous free flap was
performed with anastomosis of large vessels with the temporal artery.
In all cases (100%), reconstruction was successful and the graft survived,
providing skin coverage over orbital bone.
Conclusion. Radical exenteration for extensive orbital malignancies can
lead to large open defects that require free myocutaneous flaps from the
arm or leg with microvascular anastamosis.
Financial disclosure. None
243 ECp107
A HUGE OCULAR ADNEXAL MALT LYMPHOMA WHICH
AROSE FROM IGG4 RELATED ORBITAL LESION
Koh-ichi Ohshima (koh-1125@po6.oninet.ne.jp)
Section of Ophthalmology, Okayama Medical Center, Japan
Purpose. For the treatment of the IgG4 related orbital disorders, steroid
therapy is the first choice. On the other hand, ocular adnexal MALT
lymphoma responds well to the radiation therapy. We should discuss
how to treat MALT lymphoma which arose from IgG4 related orbital
lesion.
Methods. A 71-year-old woman visited our department for the treatment
of eyelid swelling of the left eye which had been aggravating for two
years. The left upper and lower eyelids were very swollen and the left
eyeball had atrophied. The abnormal mass of left eyelid, the swelling of
extra ocular muscle and the enlargement of major lacrimal gland were
shown by orbital MRI. Auto-antibodies related to the thyroid gland were
negative, and serum IgG4/IgG was 450/1844. The incisional biopsy of
eyelid tumor revealed it to be MALT lymphoma with many IgG4 positive
plasma cell infiltration.
Results. The eyelid tumor was surgically removed and the deformity
of the eyelid decreased. To prevent re-growth of the tumor, radiation
therapy of 30Gy was added to the left orbit. The patient has been
carefully followed up for one year.
Conclusions. Excision and radiation therapy performed to treat a huge
ocular adnexal MALT lymphoma which arose from an IgG4 related orbital
lesion, with a good result.
Financial disclosure. None
223 ECp108
HIGH-RESOLUTION ULTRASONOGRAPHY IN THE
DIAGNOSIS OF ORBITAL MALIGNANT LYMPHOMA
S.V. Saakyan, A.G. Amiryan, V.R. Alikhanova (svsaakyan@yandex.ru)
Moscow Helmholtz Research Institute of Eye Diseases
Purpose. To characterize the diagnostic criteria of orbital malignant
lymphoma through the use of complex ultrasonography techniques.
Methods. 21 patients with orbital lymphoma (mean age 55.2±16 years)
were surveyed. The examination was performed on an ultrasonic system
with volume-scanning, Voluson® 730 PRO (Kretztechnik’’s – GE Medical
System, Austria). The Duplex scanning included B-mode examination,
color Doppler Imaging (CDI), spectral Doppler analysis, and threedimensional
reconstruction. In all cases, the diagnosis of lymphoma
was morphologically verified.
Results. The lymphoma was localized in the lacrimal glands in 11

patients. The isolated involvement of extraocular muscles was
revealed in 4 patients. In 4 other cases the tumor was identified
behind the sclerouveal ring surrounding the optic nerve. In 2 cases
the tumor occupied all retrobulbar space. In B-mode examination
the lymphoma presented as a complex formation, including small
hyperechogenic inclusions and thin septations in the tumor. The
acoustic density of the tumor estimated with densitometry was -44
on average.
Mixed arterio-venous flow imaging of tumor vessels demonstrated
the average systolic flow velocity equaling 15,67cm/c; the indicators
of resistance averaged RI=0,62±0,1 and PI=1,06±0,3. “Large” feeding
arteries with the lower resistance indexes and diastolic flow were
revealed in 50 % of cases.
Conclusions. In concert with existing Methods, the echographic
criteria presented enhance the diagnostic capabilities of malignant
lymphoma of the orbit and allow for more precise determination of
the extent of surgical intervention.
Financial disclosure. None
1752 ECp109
CONJUNCTIVA MALIGNANT MELANOCYTIC TUMORS.
RECURRENCE AND SURVIVAL RATE IN 15 PATIENTS
FROM CHILE
Maria E Manquez1, Pablo Vigorena2, Bruno Nervi3, Pablo Zoroquiain3,
Jeannie Slater4, Arturo Espinoza5 (m_manquez@yahoo.com)
1. Clinica Oftalmlogica Pasteur. Santiago, Chile
2. Hospital Padre Hurtado, Santiago, Chile
3. Universidad Catolica de Chile
4. Hospital Militar Santiago, Chile
5. Citolab, Santiago, Chile
Purpose. To describe the clinical features of affected patients,
regarding age, sex, systemic condition, prior treatment, and to
describe the clinical features of the tumor, extension, presence of lid
/ orbital invasion, and to report recurrence, metastasis and mortality
rate at short term follow up.
Methods. Review of 70 charts of patients with conjunctiva melanocytic
lesions, 15 of them presented malignant tumors. Time of follow up was
36 months ( 12-72).
Results. Regarding patients: the age of presentation was 56 yo ( 33
-88), 74% were female, 80% wiith dark hair, borwon iris. None were
immunosuppressed.
Regarding tumors: 74% of cases presented melanoma with pam with
atypia, 26% with pam with severe atypia. 70% of cases had at least 1
prior recurrence.
Orbital involvement was observed in 20% of the cases
Recurrence after treatment was observed in 8% of pts with prior
surgery and 0% of those with no prior treatment.
The survival rate was 94%.
Conclusions. Mlaignant melanocytic are observed no only in caucasians
but also in hispanic patients. Our patients seems to be younger at the
time of presentation. Females are more often affected.
Most of our patients are referred late, after at least 1 surgery.
Those patients with no prior surgery, present good outcome with no
recurrence at short term follow up.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
85
66 ECp110
LYMPHOPROLIFERATIVE TUMORS OF THE LACRIMAL
GLAND: ANALYSIS OF CLINICAL FEATURES AND
SYSTEMIC INVOLVEMENT
Hakan Demirci, Shivani Gupta, Carol L. Shields, Jerry A. Shields, Victor
M. Elner,
(hdemirci@med.umich.edu)
W. K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI
Oncology Service, Wills Eye Institute, Philadelphia, PA
Purpose. The lacrimal gland is an only ocular adnexal tissue that
contains native lymphocytes. Despite its unique property, isolated
primary involvement of the lacrimal gland is rare in lymphoproliferative
tumors of the ocular adnexa. There is no information about the risk
for systemic lymphoma in the patients with isolated lacrimal gland
lymphoproliferative tumors.
Methods. We retrospectively reviewed clinical features and treatment
method of 40 patients with isolated lacrimal gland tumor. Data from 33
patients without systemic disease at presentation were analyzed for
their impact on the occurrence of systemic lymphoma.
Results. At presentation, tumor was unilateral in 22 (55%) patients and
bilateral in 18 (45%). Of 40 patients, 20 (50%) patients had marginal
zone B-cell lymphoma, 13 (33%) patients had follicular lymphoma, 3
(8%) patients had mantle cell lymphoma and 2 (5%) patients had diffuse
large B-cell lymphoma. Thirty-five (88%) patients were managed with
external beam radiotherapy, 2 (5%) patients were treated with systemic
chemotherapy and 2 (5%) patients were managed with excisional
biopsy.
Systemic lymphoma was detected in 11 (28%) patients. Systemic
involvement was diagnosed before the lacrimal gland tumor in 2 (5%)
patients, at the time of diagnosis of the lacrimal gland tumor in 5 (13%)
patients, and subsequent to the diagnosis of the lacrimal gland tumor in 4
(10%) patients. Of 4 patients in whom systemic lymphoma subsequently
developed, 3 (75%) patients had follicular lymphoma and 1(25) patient
had diffuse large B- cell lymphoma. Using Kaplan-Meier estimates of 30
patients without systemic involvement, systemic lymphoma developed
in 16% at 3 and 5 years, and 58% at 10 years.
Conclusions. The majority of lacrimal gland lymphoproliferative tumors
were either marginal zone B-cell or follicular lymphoma. In patients
with lacrimal gland lymphoproliferative tumor alone at presentation,
systemic lymphoma eventually developed in 16% at 5 years and 58%
at 10 years. Development of systemic lymphoma was associated with
follicular or diffuse large B-cell lymphoma of the lacrimal gland.
Financial disclosure. None
224 ECp111
IMMUNOHISTOCHEMICAL AND MOLECULAR STUDY
OF OPTIC PATHWAY GLIOMAS
Charles G. Eberhart, J. Douglas Cameron, Elizabeth J. Rushing, Matthias
Karajannis, Fausto J. Rodriguez, (ceberha@jhmi.edu)
Johns Hopkins University, Baltimore, MD; Armed Forces Institute of
Pathology, Washington, DC; New York University, NYC
Purpose. Optic pathway gliomas (OPG) represent a specific subtype of
astrocytoma with unique clinicopathologic and biological properties.
OPG may occur in the setting of NF1 syndrome or sporadically, and
at the pathologic level are pilocytic astrocytomas. Recent studies

have highlighted a role for specific cell signaling pathways and the
tumor microenvironment in the biology of this tumor. In this study we
performed immunohistochemical studies in a relatively large series of
OPG using tissue microarrays.
Methods. Tumors from 59 patients with a median age of 9 years (range
3 mo-66 years; 33 F,26 M) were tested using formalin-fixed paraffin
embedded material in tissue microarrays. Immunohistochemistry
was performed using antibodies recognizing mTOR pathway signaling
components (total mTOR protein, Phospho-4E-BP1, PhosphoeIf4G,
phosho-S6), microglia (CD68), markers of senescence (p16),
and mutant IDH1 protein. Scoring was performed using a 4-tiered
semiquantitative scale (0-4+). IDH1 was scored as positive or negative.
Immunohistochemistry for GFAP was performed to evaluate for adequacy
of immunoreactivity.
Results. Immunohistochemical stains demonstrated frequent staining
for mTOR pathway components, including moderate (2+) to strong (3+)
staining for total mTOR (50%), phospho-4E-BP1 (42%), phospho-eIf4G
(75%), and phospho-S6 (70%). Moderate to marked numbers of CD68
positive microglia were identified in 44% of cases. Moderate to diffuse
nuclear immunoreactivity for p16 was identified in 59% of cases. All
cases (except for 1) were immunonegative for IDH1 mutant protein.
Conclusions. OPG demonstrate increased total/activated levels of mTOR
signaling components supporting an important role for this pathway
in its biology. In addition, increased numbers of CD68+ microglia and
markers of senescence (p16) were identified. These findings merit
further study and correlation with additional molecular markers to
evaluate their clinical and biological significance.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
86
Other Intra-ocular Tumors Posters
131 OTp101
PHOTODYNAMIC THERAPY FOR ACQUIRED ASTRO-
CYTOMA
Cinzia Mazzini, Maria Carla Donati, Giulia Pieretti, Ugo Menchini (cinzia.
mazzini@unifi.it)
Department of Specialistic Surgery Sciences, Eye Clinic, University of
Florence, Florence, Italy.
Purpose. Retinal astrocytomas are glial tumours of the RNFL arising from
retinal astrocytes, often associated with systemic disorders ( tuberous
sclerosis or neurofibromatosis) but they can also be found on retinal
examination as isolated tumour. We report the case of epipapillary
retinal astrocytoma associated with serous retinal detachment treated
with photodynamic therapy (PDT).
Methods. A 32-years old man was referred for an elevated whitish
epipapillary lesion associated with retinal detachment. He had a history
of multicellular carcinoma and melanocytic nevus of the scapular region.
Visual acuity was 20/20 in both eyes. Fluorescein angiography revealed
a hyperfluorescence of the lesion; B-scan showed a solid epipapillary
tumour with high reflectivity, posterior shadowing, and a contiguous
serous retinal detachment. OCT showed a solid elevation of the retinal
layers associated with raised neuro-epithelium at the edge of the lesion.
The clinical picture was consistent with “acquired astrocytoma”.
Four years later, the patient complained of a severe visual loss in left
eye. BCVA was 20/50 and fundus examination, echography, fluorescein
angiography and OCT showed an enlargement of the lesion and retinal
detachment, with macular exudation. PDT with verteporfirin was performed.
Results. Three months after treatment visual acuity remained stable;OCT
and echography revealed a decrease of subretinal fluid. One year later
BCVA was 20/30, and retinal detachment was disappeared.
Conclusions. PDT with verteporfin can induce regression of aggressive
retinal astrocytomas and may prevent their progression to total retinal
detachment and enucleation. In selected severe cases, PDT may be
considered a first-line treatment for acquired astrocytomas.
Financial disclosure. None
530 OTp102
MANAGEMENT OF PERIPAPILLARY HEMANGIOMA IN
PEDIATRIC VHL DISEASE
Prithvi Mruthyunjaya, MD (mruth001@mc.duke.edu)
Ocular Oncology and Vitreoretinal Surgery Services, Duke Eye Center,
Durham, NC
Purpose. To describe the management of a peripapillary retinal
hemangioma in a pediatric patient with von-Hippel-Lindau disease and
to describe the early development of epiretinal proliferations in this
disease.
Methods. Case report of a 2 yo male with a strong family history of VHL
disease who presents with a new peripapillary hemangioma associated
with a dome-shaped epimacular elevation.
Results. Intraoperative photodynamic therapy was performed with
verteporfin and intravitreal Avastin on 2 occasions with subsequent
inactivation of the peripapillary hemangioma. The macular elevation
was likely due to vitreous traction on the macular hyloid bursa with

exudative fluid from the hemangioma. Collapse of this structure may be
the origin of an epiretinal membrane.
Conclusions. Intraoperative PDT therapy is an option in pediatric VHL
patients with peripapillary hemangiomas. Technique for this procedure
performed under general anesthesia will be discussed.
Financial disclosure. None
105 OTp103
MEK INHIBITOR-ASSOCIATED SEROUS RETINOPATHY
- CLINICAL FEATURES OF A NEW RETINAL DISORDER
Scott C. N. Oliver, Raul Velez Montoya, Wells Messersmith, Jeffrey L
Olson, Naresh Mandava (scott.oliver@ucdenver.edu)
Departments of Ophthalmology and Medical Oncology, University of
Colorado School of Medicine, USA
Purpose. MAPK/ERK kinase (MEK) inhibitors are a promising class
of antineoplastic agents undergoing human clinical trial. This report
describes new findings of serous retinopathy and outer retinal thickening
not previously reported with these agents
Methods. Retrospective review
Results. Three patients with metastatic cancer had multiple central
scotomas 1,3, and 14 days after study drug initiation. Subneurosensory
fluid (SRF) was visible clinically and on optical coherence
tomography(OCT). Additionally, OCT identified diffuse outer retinal
thickening in areas not associated with fluid. FA and ICG angiography
did not show leakage. Findings resolved with drug discontinuation.
Conclusions. MEK inhibitor associated serous retinoapathy consists
of rapid onset localized SRF and diffuse outer retinal thickening. It
resembles, but is distinct from CSR, and requires further investigation.
Financial disclosure. Dr. Oliver is a consultant with Array Bioscience, Inc.
1450 OTp104
BENIGN TUMORS OF CILIARY BODY IN KOREAN
PATIENTS
Christopher Seungkyu Lee1A, Hee Jung Kwon1A, Hyae Min Jeon1B, Min
Kim1A, Sung Chul Lee1A (sklee219@yuhs.ac)
(A) Department of Ophthalmology,
(B) Department of Pathology,
(1) Yonsei University College of Medicine, Seoul, Korea
Purpose. To evaluate clinical features, management, histopathology,
and prognosis of benign ciliary body tumors in Korean patients.
Methods. A retrospective medical chart review was conducted on various
benign tumors of ciliary body that were evaluated by the authors between
2006 and 2011.
Results. All 8 patients underwent transscleral local resection of ciliary
body tumors, which were pathologically confirmed to be benign. Of 8, 3
were diagnosed with adenoma of nonpigmented ciliary body epithelium
(2 men, 1 woman; mean age 35 years; mean initial and final visual acuities
(VAs) 20/25 and 20/32 over 2 years), 1 was adenoma of pigmented ciliary
body epithelium (woman; 83 years; mean initial and final VAs 20/200
and light perception over 1 month), 2 were melanocytoma (2 men; mean
age 40 years; mean initial and final VAs 20/25 and 20/25 over 2 years),
1 was schwannoma (woman; 30 years; initial and final VAs 20/100 and
detecting hand movement over 5 years), and 1 was leiomyoma (woman;
46 years; initial and final VAs 20/400 and 20/80 over 1.5 years).
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
87
Conclusions. Various benign tumors can be found in the ciliary body
that should be differentiated from malignant tumors, especially ciliary
body melanoma. Benign ciliary body tumors are rare and each type of
tumor has its characteristic clinical and histopathological features that
can suggest the diagnosis. In the management of these tumors, local
resection, rather than enucleation, should be considered first as the
treatment of choice. The visual prognosis was fair without any systemic
morbidity.
Financial disclosure. None
321 OTp105
TREATMENT OF SEROUS MACULAR DETACHMENT
ASSOCIATED WITH CIRCUMSCRIBED CHOROIDAL
HEMANGIOMA
Sung Chul Lee, Hee Jung Kwon, Min Kim, Christopher Seungkyu Lee
(sunglee@yuhs.ac)
Department of Ophthalmology, Institute of Vision Research, Yonsei
University College of Medicine, Seoul, Korea
Purpose. To evaluate the effect of transpupillary thermotherapy
(TTT) and intravitreal injection of bevacizumab (IVB) on serous
macular detachment (SMD) associated with circumscribed choroidal
hemangioma (CCH).
Methods. We retrospectively reviewed the records of CCH patients
treated by TTT and/or IVB to reduce SMD. We assessed changes in
visual acuity (VA), resolution of SMD and central fovea thickness (CFT) by
optical coherence tomography (OCT).
Results. Of 8 patients treated with TTT, 5 patients showed complete
resolution of SMD in OCT with improvement of median LogMAR VA from
0.7 to 0.22 (p=0.042). These 5 patients showed no recurrence of SMD
for a mean duration of 51.8 months, but SMD finally recurred in 4 of
them.
Of 9 patients treated with IVB, 5 patients showed resolution of SMD with
a decrease of median CFT from 514 µm to 330 µm (p=0.043). Of these 5
patients, 2 patients showed recurrence of SMD after 6.2 months.
In TTT group, 5 patients (3 patients in complete SMD resolution group,
2 patients in persistent SMD group) received additional IVB and 4 of
them also had cystoids macular edema (CME) with SMD before IVB.
All the patients showed persistent CME after IVB despite the complete
resolution of SMD in two of them.
Conclusions. In some patients with SMD in CCH, both TTT and IVB may
be used effectively. However, the duration of treatment effectiveness
appears to be longer in TTT than that in IVB. IVB for recurred SMD after
TTT Results in variable outcome but IVB had no effect on CME.
Financial disclosure. None
2132 OTp106
INTRAOCULAR RELAPSE OF MULTIPLE MYELOMA
RESPONSIVE TO BORTEZOMIB
Tero Kivelä (tero.kivela@helsinki.fi)
Department of Ophthalmology, Helsinki University Central Hospital,
Helsinki, Finland
Purpose. To report complete response to systemic bortezomib of a
relapse of multiple myeloma in the iris.
Methods. A 60-year-old man was diagnosed with multiple myeloma,

IgG λ type (stage IIIA) in 2000. He was first managed with vincristine,
adriamycin and dexamethasone, resulting in partial response (PR), and
then with cyclophosphamide and dexamethasone, resulting in complete
response (CR), followed by autologous stem cell transplantation.
No M-component was detected until May 2008, when a solitary
plasmocytoma of the humerus appeared and was managed with
thalidomide. Soon thereafter, a new, diffusely infiltrating unilateral iris
lesion developed.
Results. At diagnosis the patient was asymptomatic with 20/20 vision,
IOP 14 mmHg, and cells in the anterior chamber. The iris was diffusely
reddish with a circle of pigment epithelial cysts around the pupillary
margin. The fundus was normal. He received two courses of bortezomib,
a reversible proteasome inhibitor, with dexamethasone. After the 1st
course, the cells and the redness of the iris disappeared. Two months
after the 2nd course of bortezomib, while on lenalidomide maintenance
therapy, he continued to be asymptomatic with an IOP of 14 mmHg and
a normal iris.
Conclusions. Bortezomib was effective in eradicating an intraocular
replase of multiple myeloma in a patient who previously had received
several types of conventional chemotherapy.
Financial disclosure. None
1923 OTp107
TUBEROUS SCLEROSIS COMPLEX: CHARACTERIZA-
TION OF OCULAR MANIFESTATIONS AND CORRELA-
TIONS WITH SYSTEMIC DISEASE
M.E. Turell1, E.I. Traboulsi1, A. Gupta2 , A.D. Singh1 (turellm2@ccf.org)
1. Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, OH
2. Department of Pediatric Epilepsy, Cleveland Clinic Foundation,
Cleveland, OH
Purpose. To evaluate genotype/phenotype correlations in individuals
with astrocytic hamartoma (AH) and retinal achromic patch (AP) in the
setting of tuberous sclerosis complex (TSC).
Methods. Participants included 132 patients from the Cleveland Clinic
Foundation Tuberous Sclerosis Program (CCF-TSCP) and 907 patients
from the TSC Alliance (TSC-A). Gender, age at TSC diagnosis, presence
of TSC1 or TSC2 mutations, ophthalmic examination, and systemic
manifestations were analyzed.
Results. No difference was found in the prevalence of AH in the CCF-
TSCP (36.1%) and TSC-A (34.1%) groups (p = 0.743). AH were bilateral
in 43.3% and 18.1% (p = 0.009) and multiple in 40.0% and 15.3% (p =
0.008) in the CCF-TSCP and TSC-A groups respectively. In the CCF-TSCP
group, AP was observed in 12.0% of patients (40.0% bilateral, 50.0%
multiple). The presence of retinal features was associated with giant cell
astrocytoma (37.1% vs 14.6%; p = 0.018), renal angiomyolipoma (60.0%
vs 27.1%; p = 0.003), cognitive impairment (77.1% vs 43.8%; p = 0.002),
and epilepsy (91.4% vs 70.8%; p = 0.022) in those with and without
retinal findings respectively. In patients with retinal findings in the CCF-
TSCP and TSC-A groups, mutations in TSC2 were more frequent than
in TSC1, 3.3 times and 5.8 times, respectively. In those without retinal
findings; the relative rates were 0.67 times and 2.3 times, respectively.
Conclusions. Individuals with retinal findings are more likely to have
subependymal giant cell astrocytomas, renal angiomyolipomas,
cognitive impairment and epilepsy. TSC2 mutations are more frequent
in patients with retinal findings than in those without.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
88
Support: Research to Prevent Blindness
Challenge Grant, Department of
Ophthalmology, Cleveland Clinic Lerner
College of Medicine
248 OTp108
INTERLEUKIN LEVELS IN AQUEOUS OF UNTREATED
AND TREATED EYES WITH VITREORETINAL LYMPHO-
MA
Jose S. Pulido, MD, Joseph Balsanek, Brian Peters, Melissa Snyder, PhD
(pulido.jose@mayo.edu)
Department of Ophthalmology, Department of Laboratory Medicine and
Pathology
Purpose. To determine the levels of interleukin levels in aqueous
samples taken at the time of intravitreal treatment in patients with
vitreoretinal lymphoma
Methods. Samples at the time of paracentesis were immediately placed
on ice and then ELISA for interleukin levels were performed
Results. 81 samples were tested. IL 10 levels had a mean of 62 with
range of 0.69-624 pg/ml. IL-6 had a mean 123 pg/ml with range from
1.67-960 pg/ml. Other levels that were elevated included were MCP-
1,and IL-1ra.
Conclusions. There are marked changes in interleukin levels depending
upon treatment levels. IL-10 aqueous levels is a good measure of
treatment response to intravitreal chemotherapeutic agents
Financial disclosure. None
304 OTp109
INTRAVITREAL RITUXIMAB FOR PRIMARY INTRAOCU-
LAR LYMPHOMA (PIOL)
V. Kakkassery1, G. Willerding1, K. Jahnke2, A. Korfel2, U. Pleyer1, N.
Stübiger1, A.M. Joussen1 (vk@charite.de)
1. Department of Ophthalmology, Charité, Berlin, Germany
2. Department of Hematology and Oncology, Campus Benjamin Franklin,
Charité, Berlin, Germany
Purpose. PIOL is a rare manifestation of primary central nervous system
lymphoma (PCNSL). Due to its rarity, the optimal treatment of this
condition has not yet been defined thus far. The chimeric monoclonal
CD20 antibody rituximab offers a new intravitreal treatment option for
PIOL. Here, we report on the clinical course after repeated intravitreal
injections of rituximab for PIOL.
Methods. Diagnosis of PIOL was confirmed by clinical investigation
and vitreous biopsy in three cases. Two patients were pretreated with
systemic chemotherapy for their PCNSL (ifosfamide or MTX). Ocular
clinical findings (visual acuity, intraocular pressure, vitreous haze, extent
of tumor) were recorded before and after rituximab therapy. Intravitreal
1mg/0,1mL rituximab injections were conducted in accordance with the
German Ophthalmic Society guidelines for intravitreal injections. Data
were implemented in a nationwide open registry for PIOL supported by
the German Federal Ministry of Education and Research.
Results. Five eyes from 3 patients received a minimum of one to a

maximum of 7 rituximab injections. After a follow up of 4 -30 months, we
observed a significant reduction of vitreous haze in all eyes (measured
by the Nussenblatt classification), a preserved constant visual acuity
or improvement (two eyes: finger count to 20/32; 20/40 to 20/32) and
tumor remission. All patients reported about a reduction of discomfort
after therapy. No intraocular adverse effects have been observed.
Conclusions. In our case series, we demonstrated improvement of clinical
symptoms and signs in PIOL and, to some extent, visual acuity without
adverse intraocular side effects after intravitreal rituximab injections.
Further long-term studies are necessary to further investigate local and
systemic effects and possible side effects after rituximab therapy for
intraocular lymphoma.
Financial disclosure. None
EYELID, CONJUNTIVA & ORBIT
Abstract Posters
89

8.30-9.00 Poster Presentations UMp101-113
(Moderators: A. Singh, D. Pelayes)
9.00-10.20 Papers
(Moderators: N. Bornfeld, M.J. Jager)
2253 UM1
PRELIMINARY STUDY OF VASCULAR FLOW IN
CHOROIDAL TUMORS AND PSEUDOTUMORS
C.M. Gentile, M. Faria Dovasio , Lucila Tajtelbaum, Atilio Lombardi , J.
Oscar Croxatto
622 UM2
AUSTRALIA AND NEW ZEALAND STUDY OF PDT IN
CHOROIDAL AMELANOTIC MELANOMA (ANZSOPI-
CAM) - TWO YEAR RESULTS
William Glasson, William G. Campbell, Sid Finnigan, Michael Giblin,
Peter Hadden, Timothy Isaacs, John D. McKenzie, James Muecke, Tanya
M. Pejnovic
1618 UM3
PHOTODYNAMIC THERAPY AS ADJUVANT TREAT-
MENT FOR AMELANOTIC CHOROIDAL MELANOMA
DEBULKING
Maria A. Blasi, Monica M. Pagliara, Andrea Scupola, Carmela G. Caputo,
Emilio Balestrazzi
1413 UM4
MACROPHAGE INFILTRATION IN PREVIOUSLY-IRRA-
DIATED UVEAL MELANOMA
M.J. Jager, T.H.K. Vu, I.H.G. Bronkhorst, M. Versluis, M. Marinkovic, S.G.
van Duinen, G.P.M. Luyten
2131 UM4
FINE NEEDLE ASPIRATION BIOPSY OF UVEAL MELA-
NOMA : EVALUATION OF A CALIBRATED NEEDLE
David E. Pelayes, Jorge O. Zárate, Charles V. Biscotti, Arun D. Singh
56 UM5
25G/23G TRANSRETINAL BIOPSY IN UVEAL TUMOURS
N. Bornfeld, M. Gök, E. Biewald, M. Freistühler, M. Zeschnigk
1601 UM7
UVEAL TRACT MELANOMA: CORRELATION OF GENET-
IC HETEROGENEITY WITH MACROSCOPIC MORPHOLOGY
Vasilios P. Papastefanou, Ajay Patil, Marianne Grantham, Mandeep S.
Sagoo, Victoria M.L. Cohen
49 UM8
DEVELOPMENTS IN PREDICTING METASTASIS FROM
CHOROIDAL MELANOMA
B.E. Damato, A. Eleuteri, A.F. Taktak, S.E. Coupland
Thursday November 17, 2011
UVEAL MELANOMA
Program
91
444 UM9
FISH-BASED PROGNOSTICATION OF UVEAL MELA-
NOMA
M. Turell, R. Tubbs , C. Biscotti, L. Schoenfield, Y. Sun G. Bebek , Y.
Saunthararajah, P. Triozzi, A. Singh
1819 UM10
FLUORESCENCE IN-SITU HYBRIDIZATION VS MULTI-
PLEX LIGATION-DEPENDENT PROBE AMPLIFICATION
FOR UVEAL MELANOMA PROGNOSTICATION: IN-VI-
VO COMPARATIVE RESULTS
Raffaele Parrozzani, Elisabetta Pilotto, Alessia Dario, Giacomo
Miglionico, Edoardo Midena
10.20-10.50 BREAK
10.50-11.20
THE STALLARD MEDAL
Prof. ENRIQUE S. MALBRAN
ANTERIOR SEGMENT SURGERY OF INTRAOCULAR TUMORS
11.20-12.00 Papers
(Moderator: J. Augsburger, B. Damato)
2331 UM11
UVEAL MELANOMA: AN ANALYSIS OF CELLULAR FEA-
TURES AND COMPARISON TO MONOSOMY 3 STA-
TUS.
C.V. Biscotti, A. Lott-Limbach, R.R. Tubbs, M.E. Turell, Y. Sun, A.D.
Singh
2321 UM12
RETROSPECTIVE ANALYSIS OF 700 FINE-NEEDLE
ASPIRATION BIOPSIES OF SOLID INTRAOCULAR
TUMORS: CHANGING INDICATIONS DURING LONG-
TERM EXPERIENCE
Zélia M. Corrêa, James J. Augsburger
204 UM13
PROSPECTIVE EVALUATION OF A GENE EXPRESSION
PROFILE PROGNOSTIC ASSAY FOR UVEAL MELANOMA IN
514 PATIENTS
J. William Harbour, Michael D. Onken, Lori A. Worley, James J. Augsburger,
Zelia M Correa, Devron H. Char, Eric Nudleman, Thomas M. Aaberg,
Jr., Michael M. Altaweel, David S. Bardenstein, Paul T. Finger, Brenda
L. Gallie, George J. Harocopos, Peter G. Hovland, Hugh D. McGowan,
Tatyana Milman, Prithvi Mruthyunjaya, E. Rand Simpson, Morton E.
Smith, David J. Wilson, William J. Wirostko

153 UM14
PHENO-GENOTYPIC IDENTIFICATION OF CIRCULATING
TUMOR CELLS IN UVEAL MELANOMA
Cinzia Mazzini, Pamela Pinzani, Francesca Salvianti, Daniela Massi,
Giulia Pieretti, Mario Pazzagli, Daniela Bacherini, Ugo Menchini
12.00-12.30 Poster presentations UMp114-126
(Moderators: J.W. Harbour, G. Chantada)
12.30-14.00 LUNCH
14.00-15.40 Papers
(Moderators: T. Kivela, M. Materin)
1245 UM15
PROSPECTIVE FOLLOW-UP STUDY IN HIGH-RISK
UVEAL MELANOMA (UM) PATIENTS: FINAL RESULTS.
S. Piperno-Neumann, V. Servois, P. Mariani, J. Couturier, C. Plancher, C.
Levy-Gabriel, L. Lumbroso-Le Rouic, R. Salmon, B. Asselain, L. Desjardins
3 UM16
PERSONALIZED TARGETED THERAPY FOR UVEAL
MELANOMAS HARBORING GNAQ OR GNA11
MUTATIONS
Vasiliki Poulaki, Sue Anne Chew, Bin He, Nicholas Mitsiades
132 UM17
RATIONALE, STUDY DESIGN AND ACCRUAL STATUS
OF A RANDOMIZED PHASE II STUDY OF AZD6244,
A MEK INHIBITOR, VS TEMOZOMJDE IN ADVANCED
UVEAL MELANOMA
Richard D. Carvajal, Jedd D. Wolchok, Paul B. Chapman, Mark Dickson,
Mark Bluth, Grazia Ambrosini, Brian Marr, Murk Heinemann, Annie
Fusco, Marta Nalysnyk, Natasha Martin, Austin Doyle, Boris Bastian,
David Abramson, Gary K. Schwartz
2212 UM18
A CLINICAL PROBE FOR TRANSSCLERAL OPTICAL
SPECTROSCOPY OF INTRAOCULAR TUMOURS
J. Krohn, P. Svenmarker, C.T. Xu, S. Andersson-Engels
300 UM19
OUR EXPERIENCE WITH TRANSPUPILLARY THERMO
THERAPY FOR SUSPECTED OR SMALL CHOROIDAL
MELANOMAS
Mordechai Rosner, Iris Moroz , Josef Moisseiev, Victoria Vishnevskia-Dai
1842 UM20
RESECTION OF IRIS TUMOURS : INTERNAL APPROACH
Arun D. Singh
UVEAL MELANOMA
Program
92
1917 UM21
PLAQUE RADIOTHERAPY TREATMENT WITH RUTHE-
NIUM-106 FOR IRIS MALIGNANT MELANOMA
Amit K. Arora, Maria Tsimpida, Victoria M.L. Cohen, John L. Hungerford
1849 UM22
RUTHENIUM BRACHYTHERAPY WITH THE 10 MM
PLAQUE FOR SMALL POSTERIOR CHOROIDAL
MELANOMAS
Tero Kivelä, Susanna Salkola, Sebastian Eskelin, Jorma Heikkonen
63 UM23
REGRESSION OF UVEAL MELANOMA FOLLOWING
IODINE 125 PLAQUE RADIOTHERAPY: PREDICTIVE
FACTORS AND CORRELATION WITH METASTASIS
Hakan Demirci, Fiorella Saponara, Adam Khan, Leslie Niziol, Grant
Comer, David Musch
1802 UM24
TUMOUR REGRESSION AFTER BRACHYTHERAPY
FOR UVEAL MELANOMA: ASSOCIATION WITH
SURVIVAL
M.M. Rashid, T. Kivelä
2310 UM 25
RESULTS OF VITREORETINAL SURGERY FOLLOWING
A COMPLICATED COURSE AFTER PLAQUE
BRACHYTHERAPY OF MALIGNANT MELANOMA OF
THE UVEA
C. Metz, T. Gkika, W. Sauerwein, N. Bornfeld
2230 UM 26
LONG-TERM RESULTS AFTER ENDORESECTION OF
LARGE UVEAL MELANOMAS WITH PRETREATMENT
BY SINGLE-DOSE STEREOTACTIC IRRADIATION AND
ADJUVANT BRACHYTHERAPY
E. Biewald, H. Lautner, M. Freistühler, M. Gök, W. Sauerwein, GA
Horstmann, N. Bornfeld
15.40-16.10 BREAK
16.10-17.30 Papers Uveal Melanoma
(Moderators: A. Irarrazaval, E. Midena)
1447 UM27
TREATMENT OF JUXTAPAPILLARY CHOROIDAL MELA-
NOMA
Maria Tsimpida, John Hungerford, Victoria M.L. Cohen

2307 UM28
OPTIC NERVE DAMAGE AFTER IRRADIATION OF
INTRAOCULAR TUMOURS
Evangelos S. Gragoudas, Anne Marie Lane, Ivana Kim
133 UM29
PROTON BEAM RADIOTHERAPY OF PARAPAPILLARY
CHOROIDAL MELANOMA
G.D. Willerding, D. Cordini, N.E. Bechrakis, M.H. Foerster, J. Heufelder,
A.M. Joussen N. Lakotka, L. Moser
1414 UM30
HEMODYNAMIC MODIFICATIONS OF THE RETINA
AND THE CHOROÏD FOLLOWING PROTON BEAM
IRRADIATION OF UVEAL MELANOMAS
Leonidas Zografos, Ann Schalenbourg, Line Chamot
2358 UM31
PERIOCULAR TRIAMCINOLONE FOR PREVENTION
OF MACULAR EDEMA FOLLOWING PLAQUE
RADIOTHERAPY OF UVEAL MELANOMA: THREE YEAR
FOLLOW-UP
Noel Horgan, Carol L. Shields, Melissa Murphy, Arman Mashayekhi,
Pedro F. Salazar, Miguel A. Materin, Myra O’Regan, Jerry A. Shields
1825 UM32
INTRAVITREAL BEVACIZUMAB VERSUS
TRIAMCINOLONE ACETONIDE INJECTION FOR SEROUS
RETINAL DETACHMENT SECONDARY TO POSTERIOR
UVEAL MELANOMA
Edoardo Midena, Raffaele Parrozzani, Elisabetta Pilotto, Alessia Dario,
Giacomo Miglionico
UVEAL MELANOMA
Program
93
2326 UM33
INTRAVITREAL BEVACIZUMAB AS AN ADJUVANT
AGENT WHEN USED IMMEDIATELY AFTER TREATMENT
WITH PLAQUE BRACHYTHERAPY
Samuel K. Houston, Timothy G. Murray, Arnold M. Markoe, Yolanda
Pina, Christina Decatur
2040 UM34
INTRAVENOUS AND ORAL STEROID ADMINISTRATION
FOR PAIN CONTROL IN PLAQUE PATIENTS: A
RANDOMIZED TRIAL
A.C. Schefler, D.G. Gologorsky, J. Fulgueira, W. Feuer
1623 UM35
UVEAL MELANOMA AMONG FINNISH CHILDREN AND
ADOLESCENTS
Rana’a Aljamal, Tero Kivelä
406 UM 36
RESULTS OF MELANOMA BRACHYTHERAPY IN
ARGENTINA
Arturo Irarrazaval, Pablo Cazon

8.30-9.00 Poster presentations UMp101-113
(Moderator: A. Singh, D. Pelayes)
1350 Ump101
INDUCED EXPRESSION OF PIRES-EGR1-EGFP-OMI/
HTRA2 AND ITS EFFECT ON APOPTOSIS OF HUMAN
CHOROIDAL MELANOMA OCM-1 CELLS
Xufang Sun, Tian Yu, Shu Yan
2020 Ump102
THE HISTONE DEACETYLASE INHIBITOR SAHA
(VORINOSTAT) DOWNREGULATES HOMOLOGOUS
RECOMBINATION REPAIR PROTEINS AND CAUSES
APOPTOSIS IN UVEAL MELANOMA CELL
Grazia Ambrosini, Oliver Surriga and Gary K. Schwartz
1538 Ump103
AN INITIAL STUDY OF THE MIRNA EXPRESSION
PROFILE OF UVEAL MELANOMA
Wenbin Wei, Cheng Hsun Yang
1949 Ump104
GENDER DIFFERENCES AND ESTROGEN AND
PROGESTERONE RECEPTOR EXPRESSION IN UVEAL
MELANOMA
L. Schoenfield, M. Turell, P. Carver, S. Mackie, R. Tubbs, A. Singh
2320 Ump105
DOES INTERNAL BLOOD FLOW VELOCITY CORRELATE
WITH KNOWN PROGNOSTIC FACTORS FOR PRIMARY
UVEAL TRACT MELANOMA?
Amit Arora, Vasilios P. Papastefanou, Mandeep S. Sagoo, Marie Restori,
Marianne Graham, Victoria M.L. Cohen
1425 Ump106
EVALUATION OF IRIS AND CILIARY BODY LESIONS
WITH ANTERIOR SEGMENT OPTICAL COHERENCE
TOMOGRAPHY (AS-OCT) VERSUS ULTRASOUND B
SCAN
M.S. Sagoo, V. P. Papastefanou, S. Hau, S. Shah, M. Tsimpida, V.M.
Cohen
114 Ump107
Β-RADIATION BRACHYTHERAPY FOR LARGE
CHOROIDAL MELANOMA: DO WE NEED A HIGH DOSE
TO THE APEX?
M. Naseripour, K.H. Ghasemi Falavarjani, R. Jaberi, A.R. Irani, Z. Azma
UVEAL MELANOMA
Posters
94
2116 Ump108
ACCURATE ESTIMATION OF THE DOSE DISTRIBUTION
OF AN EYE IRRADIATED WITH RU/RH-106 EYE PLAQUE
Wolfgang Sauerwein, Norbert Bornfeld, Andrea Wittig, Birthe
Zimmermann, Dirk Flühs, Lorenzo Brualla
1520 Ump109
LOCAL IRRADIATION OF LARGE UVEAL MELANOMA
WITH TUMOR HEIGHT OVER 6,5 MM
Michael Freistühler, Eva Biewald, Mete Gök, Gkika Theodora, Dirk Flühs,
Wolfgang Sauerwein, Norbert Bornfeld
2332 Ump110
COMPARISON OF MATCHED GROUPS OF PATIENTS
TREATED WITH RUTHENIUM BRACHYTHERAPY
WITH SIMULTANEOUS THERMOTHERAPY OR
BRACHYTHERAPY ALONE
A.A. Yarovoy, D.A. Magaramov, E.S. Bulgakova
51 Ump111
SURGICAL ATTENUATION OF RADIATION FROM
I-125 BRACHYTHERAPY: PRELIMINARY SAFETY AND
FEASIBILITY DATA
Scott C. N. Oliver, Victor Hsu, Lucy Bollinger, Moyed Miften, Laurie
Gaspar, Philip Boyer
1110 Ump112
IMAGE-BASED TREATMENT PLANNING OF UVEAL
MELANOMA IN PROTON THERAPY
J. Heufelder, D. Cordini, N.E. Bechrakis, M.H. Foerster, W. Hinkelbein, S.
Höcht, B. Jamil, A.M. Joussen, B. Karle, R. Stark, A. Weber, G. Willerding,
L. Moser
1547 Ump113
LONG-TERM OBSERVATIONS IN SMALL, POSTERIOR
LOCATED MALIGNANT MELANOMAS OF THE CHOROID
TREATED WITH TRANSPUPILLARY THERMOTHERAPY (TTT)
B.M. Stoffelns, K. Schöpfer
Afternoon
12.00-12.30 Poster presentations Ump114-126 (moderators J.W.
Harbour, G. Chantada)
602 Ump114
CANCER RISKS FOR PATIENTS WITH MYOTONIC
DYSTROPHY
Jose Pulido, Aung Ko Win, Promilla Perattur, Christine Pulido, Noralane Lindor

UVEAL MELANOMA
Posters
1053 Ump115
CHOROIDAL NEOVASCULARIZATION SECONDARY TO
A CHOROIDAL NAEVUS: A CASE SERIES
Vasilios P. Papastefanou, Victoria M.L. Cohen, Martin Harris, Vanda
Nogueira, Richard M. Andrews, Mandeep S. Sagoo
1617 Ump116
INCIDENCE OF METASTATIC DISEASE AND
SURVIVAL OF 716 CONSECUTIVE PATIENTS WITH
POSTERIOR UVEAL MELANOMA: A RETROSPECTIVE
MONOCENTRIC REVIEW
Fatima Hammouch, Patrick De Potter, David Francart, François
Dall’Armellina, Jean-François Baurain
25 Ump117
LONG LASTING SURVIVAL OF UVEAL MELANOMA
WITH EXTRAOCULAR EXTENSION
Ignacio Zeolite, Carlos Zeolite, Juan Oscar Croxatto,
2133 Ump118
CLINICOPATHOLOGIC CORRELATIONS OF PLAQUE
BRACHYTHERAPY FAILURE IN THE TREATMENT OF
CHOROIDAL MELANOMA
Jill R Wells, Chris S Bergstrom, Qing Zhang, Hans E Grossniklaus
2038 Ump119
VALUE OF DOPPLER ANALYSIS IN THE REGRESSION
OF UVEAL MELANOMA AFTER PLAQUE
Mónica Asencio-Duran, Pilar Garcia-Raya, Pilar Moreno, Isabel
Rodriguez-Rodriguez, Eva Corredoira
65 Ump120
EVALUATION OF CHOROIDAL TUMORS BY OCT-
ENHANCED DEPTH IMAGING
Hakan Demirci, Dolly A. Padovani-Claudio, Alexis Smith, Brandon Smith,
Grant M. Comer
95
1608 Ump121
HISTOPATHOLOGIC FINDINGS IN EYES WITH
CHOROIDAL MELANOMA TREATED WITH
BEVACIZUMAB FOR RADIATION RETINOPATHY
Hans E. Grossniklaus, Martina C. Herwig, Weiqing Gao
64 Ump122
TREATMENT OF RADIATION MACULOPATHY WITH
INTRAVITREAL INJECTION OF BEVACIZUMAB
Mosci Carlo, Francesca Nasciuti, Francesco Baldo Lanza
327 Ump123
EPIDEMIOLOGICAL ANALYSIS OF BRAZILIAN
PATIENTS WITH UVEAL MELANOMA SUBMITTED TO
PRIMARY ENUCLEATION IN A REFERRAL CENTER
Priscilla L. Ballalai, Kelcia Kieffer, Ricardo Filippo, Rafaello Salla, Maria
Cristina Martins, Márcia Lowen
2252 Ump124
UVEAL MELANOMA CLINICAL TRIALS AT MD
ANDERSON CANCER CENTER
Scott E. Woodman, Michael Tetzlaff, Xiaoxing Yu, Chandrani
Chattopadhyay, Michelle Williams, NancyPoindexter, Elizabeth Grimm,
Dan Gombos, Bita Esmaeli, Agop Bedikian, Sapna Patel
1912 Ump125
UVEAL MELANOMA: TRENDS IN INCIDENCE,
TREATMENT, AND SURVIVAL
Arun D. Singh, Mary E. Turell, Allan K. Topham
453 Ump126
FISH: MAKING HEADS OR TAILS OF TECHNIQUES
M. Turell , R. Tubbs , C. Biscotti , Y. Sun, Y. Saunthararajah, P. Triozzi,
A. Singh

2253 UM1
PRELIMINARY STUDY OF VASCULAR FLOW IN CHOR-
OIDAL TUMORS AND PSEUDOTUMORS
C.M. Gentile, M. Faria Dovasio , Lucila Tajtelbaum, Atilio Lombardi , J.
Oscar Croxatto (carolina.gentile@gmail.com)
Hospital Italiano de Buenos Aires, Argentina
Purpose. The aim of this study was to describe and analyze the
hemodynamic findings pre and post treatment in choroidal melanocytic
tumors and in pseudotumors using high resolution doppler color and
spectral ultrasound.
Methods. Twenty six patients (aged ranged from 38 and 86 years- old)
with presumed
diagnosis of choroidal tumour or pseudotumors were included. The
B-Scan
and Doppler ultrasound images were obtained with ESAOTE My Lab 70
vision,
Italia equipment. Intralesional vascularization and spectral doppler
analysis from intratumoral vascular region and from tumoral base was
performed using values of flow velocity in systole and diastole, and
resistive index (systolic-diastolic/systolic, RI).
Results. Vascular flow with low resistive index was observed in patients
with untreated choroidal melanoma. After therapy, the intratumoral
vascularization decreased and increased the RI. In three patients with
treated melanomas, intratumoral vascularization in association with
tumoral growing was observed. There was no tumoral vascularization
in patients with choroidal nevus except for the base (choroid). Doppler
ultrasound in patients with media opacity and presumed diagnosis
of choroidal haematoma versus melanoma, revealed absence of
intralesional vascularization and it showed presence of vascularization
only on its base (choroidal vessels).
Conclusions. Doppler color and spectral ultrasound is a non invasive
and useful clinical technique which may be used in differential
diagnosis between choroidal haematoma and advanced melanoma in
patients with media opacity. It could be used as an additional tool for
the differential diagnosis of choroidal nevus and melanoma and for
evaluation of effectiveness in patients with choroidal melanoma after
conservative therapy.
Financial disclosure. None
622 UM2
AUSTRALIA AND NEW ZEALAND STUDY OF PDT IN
CHOROIDAL AMELANOTIC MELANOMA (ANZSOPI-
CAM) - TWO YEAR RESULTS
William Glasson, William G. Campbell, Sid Finnigan, Michael Giblin,
Peter Hadden, Timothy Isaacs, John D. McKenzie, James Muecke, Tanya
M. Pejnovic. (glasson@terraceeyecentre.com.au)
Dr William G. Campbell is the principal investigator, other authors are
co-investigators.
Purpose. ANZSOPICAM is an investigator-initiated prospective
multicentre clinical trial of photodynamic therapy in choroidal amelanotic
melanoma. This paper summarises the first two years’’ results.
Methods. Patients presenting with posteriorly-located amelanotic
melanoma were recruited into the study. After full ocular and systemic
assessment photodynamic therapy was applied with the Zeiss Visulas
laser, using verteporfin as the photosensitiser. PDT was repeated at
UVEAL MELANOMA
Abstracts
96
three monthly intervals intil the melanoma had completely regressed.
Results. 31 patients were recruited in the first two years. Complete
regression of the melanoma has been achieved in 19 patients to date;
eight after just one treatment, five following two treatments, five
required three sessions of PDT, whilst in one patient four treatments
were necessary. The tumour in the remaining 12 patients demonstrated
a response to PDT; of these 10 are still undergoing treatment, one was
lost to follow-up and one, an 85 year old male, died of an unrelated
condition three months after his initial PDT. Vision has remained stable
or improved in all but three patients. Three participants developed
small, local recurrences that responded favourably to a further session
of PDT. So far no evidence of systemic metastatic disease has been
found in any patients.
Conclusions. The results of this study to date indicate PDT is effective
in causing regression of amelanotic melanoma, in the majority of cases
without compromising vision. The study is ongoing.
Financial disclosure. None
1618 UM3
PHOTODYNAMIC THERAPY AS ADJUVANT TREAT-
MENT FOR AMELANOTIC CHOROIDAL MELANOMA
DEBULKING
Maria A. Blasi, Monica M. Pagliara, Andrea Scupola, Carmela G. Caputo,
Emilio Balestrazzi (mariaantonietta.blasi@rm.unicatt.it)
Department of Ophthalmology, Catholic University, Rome, Italy
Purpose. To evaluate the efficacy of photodynamic therapy (PDT) as adjuvant
treatment to reduce tumour thickness before brachytherapy for amelanotic
choroidal melanoma.
Methods. Patients and Methods: Fourteen patients with amelanotic choroidal
melanoma were recruited. Inclusion criterion was diagnosis of amelanotic
choroidal melanoma based on ophthalmoscopy, ultrasonography (US),
fluorescein angiography (FA), and ICGA. All patients underwent PDT treatment
using verteporfin infused intravenously at a dose of 6mg/m2body surface
area. Five minutes after infusion, a 689nm laser was applied with a light dose
of 100 J/cm 2 at an irradiance of 600mW/cm2 over an interval of 166 seconds.
One month after PDT all patients underwent brachytherapy.
Results. One month after PDT treatment US showed reduction of tumour
height in 9 patients (64.28%) (Group A), stable thickness in 3 patients (
21.42%) (Group B) and minimal increase in thickness in 2 patients (14.28%)
(Group C). The mean tumour thickness was 5.39mm at baseline, with a
mean reduction of 25.33% in Group A; 4.60mm and no reduction in Group
B; 2.63mm with a mean increase of 6.08% in Group C. In Group A, the mean
dose of irradiation to macula and optic nerve calculated at baseline was 76.61
and 54.2 Gy, after PDT it was 43.44 and 35.05 Gy, with a decrease of 43.3%
and 35.3% respectively.
Conclusions. The goal of treatment for uveal melanoma is to achieve local
tumour control while minimizing damage to macula and optic nerve. Use of
PDT as adjuvant therapy in order to reduce tumour thickness and consequently
brachytherapy toxic effects is encouraging.
Financial disclosure. None
1413 UM4
MACROPHAGE INFILTRATION IN PREVIOUSLY-IRRA-
DIATED UVEAL MELANOMA
M.J. Jager, THK Vu, IHG Bronkhorst, M Versluis, M Marinkovic, SG van
Duinen, GPM Luyten (m.j.jager@lumc.nl)

Departments of Ophthalmology and Pathology, Leiden University
Medical Center (LUMC), The Netherlands.
Purpose. Previous studies at our department showed that uveal
melanoma with a bad prognosis contained high numbers of infiltrating
macrophages, especially of the M2 phenotype. We wondered whether
prior treatment with irradiation would affect the degree of inflammation
as displayed by the number of tumor-infiltrating macrophages and
lymphocytes.
Methods. We analyzed 46 eyes containing a uveal melanoma that were
previously treated with radioactive plaque or proton beam therapy,
and where the eye had to be enucleated due to tumor recurrence, nonresponsiveness,
or complications after irradiation. Immunofluorescence
staining was performed to determine the presence of CD68+ and
CD68+CD163+macrophages, CD3+, CD8+ and Foxp3+ regulatory T
lymphocytes. Outcomes were compared with known clinical and
histological prognostic parameters.
Results. Numbers of CD68+ and CD68+CD163+ macrophages in
secondarily-enucleated eyes varied widely, but were not related to
the reason for enucleation. When compared to primarily-enucleated
eyes, the lymphocytic infiltration was significantly (p

Purpose. To determine the presence of genetic heterogeneity (for
chromosomes 3 and 8) in uveal tract melanoma and correlate this with
tumour size and morphology.
Methods. Ninety-five patients who underwent enucleation for primary
uveal melanoma in the period of 2009-2011 were included in the study.
Tumour dimensions were preoperatively measured (height, maximal
diameter). Corresponding areas and volumes were calculated. Tumour
morphology was classified in separate subcategories (collar-stud,
dome-shaped, bilobed, multilobed, diffuse and other) through B-mode
ultrasonography.
Double blind analysis of uveal tract melanoma by fine needle aspiration
biopsy (FNAB) (from the centre of the tumour) vs punch biopsy (from
the base of the tumour) to assess the presence of genetic heterogeneity
of chromosomes 3 and 8 using the appropriate probes. Results were
correlated with tumour dimensions, tumour size according to COMS
classification and tumour morphology. In addition the yield of FNAB was
determined and similar correlations were done.
Results. Fine needle aspiration biopsy yield i.e. sufficient cells for
diagnosis was seen in 77% (73/ 95) of tumours. Yield was higher in large
tumours according to the COMS classification (83% of large melanomas).
The yield was worst for bilobed tumours (36%, 4/11).
Genetic heterogeneity (GH) between FNAB and punch biopsies was
assessed in the remaining 72 patients. GH was 4% (3 out of 72 tumours)
for chromosome 3 and 21% (15 out of 72 tumours) for chromosome 8.
Genetic heterogeneity strongly correlated with collar-stud morphology
for chromosome 3 (100%, 3 out of 3 tumours) and for chromosome 8
(53%, 8 out of 15 tumours). In addition heterogeneity for chromosome
8 strongly correlated with large melanomas, according to COMS
classification (73%, 11 out of 15 tumours)
Conclusions. Genetic heterogeneity for chromosomes 3 and 8 is
strongly associated with collar-stud lesion morphology. Heterogeneity
for chromosome 8 is correlated with large uveal melanoma.
Financial disclosure. None
49 UM8
DEVELOPMENTS IN PREDICTING METASTASIS FROM
CHOROIDAL MELANOMA
B.E. Damato, A. Eleuteri, A.F. Taktak, S.E. Coupland (bertil.damato@
gmail.com)
Royal Liverpool University Hospital; University of Liverpool
Purpose. We previously developed prognostic models using neural
networks but these were inaccurate when baseline data were incomplete.
We therefore devoped new models based on Accelerated Failure Time.
The aim of this presentation is to evaluate our improved tool in terms of
its ability to estimate survival probability after treatment of choroidal
melanoma.
Methods. Data from 3653 patients were used to generate models for
predicting all-cause mortality.
The program produced all-cause mortality curves for patients and
controls, thereby allowing risk of metastatic mortality to be estimated.
Results. The predicted morality correlated well with the observed
mortality (Kolmogorov-Smirnov statistic, 0.79. p value 0.80). The C-index
for discrimination (correlating predictions with risk factors) was 0.75 for
the clinical model and 0.79 for the laboratory model (with fewer data).
Conclusions. Our AFT model is a significant improvement over neural
networks and provides reasonably reliable prognosis relevant to
individual patients with choroidal melanoma.
Financial disclosure. None
UVEAL MELANOMA
Abstracts
98
444 UM9
FISH-BASED PROGNOSTICATION OF UVEAL MELA-
NOMA
M. Turell 1, R. Tubbs 2 , C. Biscotti 3 , L. Schoenfield 3, Y. Sun 2 , G. Bebek
4 , Y. Saunthararajah 5, P. Triozzi 6, A. Singh1 (turellm2@ccf.org)
1. Cole Eye Institute, Cleveland Clinic Foundation
2. Department of Molecular Pathology, Cleveland Clinic Foundation
3. Department of Anatomic Pathology, Cleveland Clinic Foundation
4. Center for Proteomics and Bioinformatics, Case Western Reserve
University
5. Hematologic Oncology & Blood Disorders, Cleveland Clinic Foundation
6. Solid Tumor Oncology, Cleveland Clinic Foundation, Cleveland, OH
Purpose. To compare detection of monosomy 3 in uveal melanoma
using single nucleotide polymorphism array (SNP-A) and fluorescence
in situ hybridization (FISH)
Methods. Consecutive cases of uveal melanoma treated by enucleation
from 2004 to 2010 were analyzed. DNA was isolated from fresh frozen
tissue and was analyzed by SNP-A (Illumina, San Diego, CA) and by FISH
using both centromeric (CEP3) and locus-specific (3p26) probes (Abbott
Molecular, Des Plains, IL). A total of 200 interphase cells were scored
and a cut-point of 20% was used to determine monosomy 3 status.
Results. In this series, there were 50 Caucasian patients including 28
males (56%) and 22 females (44%) with an average age at diagnosis
of 64 years. Tumor location was choroidal in 24 (48%), ciliochoroidal
in 18 (36%), and iridociliochoroidal in 8 cases (16%). Monosomy 3
was detected by either SNP-A or FISH (CEP3) in 33 cases (66%). SNP-A
detected monosomy 3 in 31 tumors (94%). CEP3 probe detected 25
cases of monosomy 3 (76%). Locus-specific FISH probe (3p26) detected
deletion in 28 of 31 cases (90%) of monosomy 3 positive tumors by
SNP-A and every case of of monosomy 3 by CEP3 probe.
Conclusions. There is a strong correlation between monosomy 3 detected
by SNP-A and by 3p26 deletion detected by locus-specific FISH probe.
Financial disclosure. This work was supported by a Falk Trust grant
and a Research to Prevent Blindness Challenge Grant, Department of
Ophthalmology, Cleveland Clinic Lerner College of Medicine.
1819 UM10
FLUORESCENCE IN-SITU HYBRIDIZATION VS MULTI-
PLEX LIGATION-DEPENDENT PROBE AMPLIFICATION
FOR UVEAL MELANOMA PROGNOSTICATION: IN-VI-
VO COMPARATIVE RESULTS
Raffaele Parrozzani1, Elisabetta Pilotto2, Alessia Dario2, Giacomo
Miglionico2, Edoardo Midena1,2 (parrozzani@libero.it)
1. GB Bietti Eye Foundation, IRCCS, Roma, Italy.
2. Department of Ophthalmology, University of Padova, Padova, Italy.
Purpose. To compare fluorescence in-situ hybridization (FISH) and
multiplex ligation-dependent probe amplification (MLPA) for uveal
melanoma prognostication.
Methods. Twenty-four patients affected by posterior uveal melanoma
and scheduled for I125 brachytherapy were included in this prospective
study. Patients underwent in-vivo 25-G transcleral FNAB (two passes)
just before applying the radioactive plaque. Sampled material underwent
both FISH and MLPA analysis using standard procedures. Follow-up was
longer than 24 months.

Results. Follow-up was 31±8 months (range, 25-42 months). FISH
analysis revealed monosomy 3 in twelve cases (50%). MLPA revealed
monosomy 3 in thirteen cases (54%) and a 3p14-q29 deletion in one
case (4%) (classified as monosomy 3 by FISH). Nine patients (41%)
developed metastatic disease during follow-up, including the case
showing monosomy 3 only by MLPA. Patient with partial chromosome 3
deletion is still alive without metastases.
Conclusions. MLPA allows to obtain more information than standard
FISH in uveal melanoma prognostication. The biological and prognostic
value of partial chromosome 3 deletion, as well as others subtle
chromosomes alterations or complex MLPA results, remains unclear.
Financial disclosure. None
2331 UM11
UVEAL MELANOMA: AN ANALYSIS OF CELLULAR
FEATURES AND COMPARISON TO MONOSOMY 3
STATUS
C. V. Biscotti1, A Lott-Limbach1, R. R. Tubbs2, M. E. Turell3, Y Sun2, A. D.
Singh3 (biscotc@ccf.org)
1. Departments of Anatomic Pathology, 2. Molecular Pathology and 3.
Ophthalmology, Cleveland Clinic, Cleveland, Ohio USA
Purpose. Uveal melanoma patients segregate into two prognostic
groups. Approximately half will eventually manifest metastases.
Unfortunately, most experts agree that the die is cast by the time of
diagnosis. We analyzed a series of uveal melanoma FNA samples to
describe the cellular features and correlate these with the results of
FISH analysis for monosomy 3.
Methods. All patients had a clinical diagnosis of uveal melanoma
based on history and physical examination including ophthalmoscopic
examination performed by one of the authors (ADS).The patients
consented to this study which was funded by the Falk Trust. FNA using
a 25G needle was performed at the time of radiation therapy plaque
placement or enucleation/excision by two of the authors (ADS and
MET). In vivo samples were obtained by transvitreal or transscleral
approach depending on tumour location. Aspirate samples were entirely
rinsed into 2-3 ml of normal saline, visually examined for adequacy,
and then entirely transferred to a cell preservative, CytoLyt (Hologic
Corp, Marborough MA). ThinPrep® processing yielded 4 alcohol fixed
Papanicolaou stained slides per case. One of the authors (CVB) analyzed
the slides for cellular features including cell type (pure epithelioid,
pure spindle, or mixed), nuclear grade (1, 2, or 3), and the presence or
absence of necrosis, tumour infiltrating lymphocytes (Tils), and melanin.
Slides with well preserved melanoma cells in sufficient numbers were
destained and analyzed for monosomy 3 by FISH. An adequate FISH
study required 200 cells. A positive result required monosomy 3 in at
least 20% of cells.
Results. Ninety patients with a clinical diagnosis of uveal melanoma
consented to the study. This included 45 men and 45 women with
ages ranging from 26 to 96 years (mean 62). Tumours involved the
choroid 61(68%) cases, ciliochoroid 21(23%) cases, iridociliary 5(6%)
cases and iris 3(3%) cases and ranged from 3 x 3 mm to 20 x 24 mm
in basal dimensions and 1.2 mm to 15 mm in height. Fifty-eight (64%)
patients had in vivo FNA either transvitreal 33 (57%) or transscleral 25
(43%).While thirty-two (36%) patients had FNA of enucleation/excision
specimens. FNA samples had diagnostic melanoma cells in 80 (89%)
cases including 68 (76%) with a 200 cell FISH count. Six patients did
not have data recorded for the specific cellular features. This yielded
62 patients for the cytology-monosomy 3 correlation. Tumour cell type
UVEAL MELANOMA
Abstracts
99
included 40 (65%) mixed and 22(35%) spindle. There were no pure
epithelioid tumours. Most (40 cases, 65%) tumours had grade 2 nuclei.
Grade 3 and grade 1 nuclei occurred in only 13(21%) and 9(15%) tumors,
respectively. Tils occurred in 18 (29%) tumours. FISH analysis identified
monosomy 3 in 28(45%) tumours. Monosomy 3 occurred significantly
more often in tumours with grade 3 nuclei (69% versus 39% in tumours
with grade 1 or 2 nuclei, p=0.0498). Monosomy 3 occurred more often
in tumours with TILs (61% versus 39% in tumours lacking TILs); this
difference is not significant (p=0.11).
Conclusions In our series most uveal melanomas had a relatively
consistent cellular appearance characterized by at least some spindle
cells, no more than moderate nuclear atypia, and melanin. In our
experience monosomy 3 occurred more often in tumours with grade 3
nuclei or Tils; however, the positive predictive value of these cellular
features is low, limiting clinical utility.
Financial disclosure. None
2321 UM12
RETROSPECTIVE ANALYSIS OF 700 FINE-NEEDLE
ASPIRATION BIOPSIES OF SOLID INTRAOCULAR
TUMORS: CHANGING INDICATIONS DURING LONG-
TERM EXPERIENCE
Zélia M. Corrêa, James J. Augsburger correazm@uc.edu
Department of Ophthalmology, University of Cincinnati College of
Medicine, Cincinnati, Ohio, USA
Purpose. Describe the changing indications for and techniques of FNAB
of intraocular tumours in a single group’s experience during a period of
over 30 years.
Methods. Retrospective records review and data analysis on 700 cases
of FNAB of a solid intraocular tumour performed by the authors during
the period 9/1980 through 6/2011.
Results. During the first 5 years, most FNABs were investigational
(including post-enucleation & post-resection to evaluated different
calibers and lengths needles, different methods of aspiration, routes
of needle passage, cellular yield, specimen processing, cytologichistologic
correlation, and provide experience with cytology of the
various types of tumours to the pathologists reading these samples).
All clinical FNABs (diagnostic or confirmatory) during this period were
performed under an IRB approved protocol in which clinical diagnostic
accuracy, cytopathologic diagnostic accuracy, complications of FNAB,
and percentage of cases in which the results of FNAB changed patient
management were evaluated. In 1985, the IRB determined that FNAB
of intraocular tumours by us no longer needed to be regarded as
investigational. During the ensuing 10 to 15 years, most FNABs we
performed were clinical diagnostic or confirmatory biopsies. Initially,
amelanotic uveal melanoma versus metastatic cancer to uvea was the
most common differential diagnosis, and later on, it became large uveal
nevus versus small uveal melanoma. Our practice relocated to a different
institution in 1999 prompting a new round of post-enucleation FNABs
to familiarize our new pathologists with the cytopathologic features of
intraocular tumours. In 2006, retrospective analysis of 25-year experience
with FNAB of clinically diagnosed melanocytic uveal tumours showed that
cytologic classification to be an independently significant prognostic
factor for metastasis and metastatic death. From that point on, all of our
FNABs on clinically diagnosed melanocytic uveal tumours have been
regarded as prognostic. In 2007, we began to collaborate in a prospective
IRB-approved multicenter (COOG) study of the prognostic significance of

gene expression profile of melanocytic uveal tumour cells obtained by FNAB.
Since then, virtually all FNABs of clinically diagnosed uveal melanomas were
simultaneously confirmatory, prognostic (cytology), and investigational for GEP
while those performed on tumours diagnosed as nevus versus melanoma were
simultaneously diagnostic, prognostic (cytology), and investigational for GEP.
In late 2009, the COOG study validated the prognostic value of GEP of large and
medium size uveal melanomas. A commercial laboratory (Castle Biosciences,
Inc.) acquired the GEP test and all biopsies of similar tumours performed since
then have been regarded as prognostic simultaneously for GEP and cytology.
Conclusions. Indications for performing FNABs on solid intraocular tumours in
this series changed substantially over the years. The FNAB method employed in
a given case depends on indication for biopsy, timing of biopsy, and specimen
size for a successful result.
Financial disclosure. Research to Prevent Blindness, New York, NY, USA, and Quest for Vision
Fund, University of Cincinnati, Cincinnati, OH. USA
204 UM13
PROSPECTIVE EVALUATION OF A GENE EXPRESSION
PROFILE PROGNOSTIC ASSAY FOR UVEAL MELANOMA
IN 514 PATIENTS
J. William Harbour, Michael D. Onken, Lori A. Worley, James J. Augsburger,
Zelia M Correa, Devron H. Char, Eric Nudleman, Thomas M. Aaberg,
Jr., Michael M. Altaweel, David S. Bardenstein, Paul T. Finger, Brenda
L. Gallie, George J. Harocopos, Peter G. Hovland, Hugh D. McGowan,
Tatyana Milman, Prithvi Mruthyunjaya, E. Rand Simpson, Morton E.
Smith, David J. Wilson, William J. Wirostko (harbour@vision.wustl.edu)
Washington University, University of Cincinnati, Tumori Foundation,
Michigan State University, University of Wisconsin, Case Western
Reserve University, New York Eye Cancer Centre, Colorado Retina
Associates, University of Toronto, New York Eye and Ear Infirmary, Duke
University, Oregon Health & Science University, Medical College of
Wisconsin
Purpose. A 15 gene expression profiling (GEP) assay was developed to assign
primary uveal melanomas to prognostic subgroups: class 1 (low metastatic
risk) and class 2 (high metastatic risk). This study evaluated the prognostic
performance of the 15-gene assay in a prospective, multicenter study.
Methods. 514 patients with uveal melanoma were enrolled in a prospective,
12-center study. Tumours were assigned to class 1 or class 2. 293 of the
samples were also analyzed for chromosome 3 status using a single
nucleotide polymorphism assay. Patients were managed for their primary
tumour and monitored for metastasis.
Results. The GEP was class 1 in 308 cases (60.0%) and class 2 in 206 cases
(40.0%). Of the class 1 cases, 308 (87.0%) were class 1A and 67 (13.0%)
were class 1B. Metastasis was detected in 2 (0.8%) class 1A patients, 7
(10.4%) class 1B, and 61 (29.8%) class 2 (P

Purpose. Despite advances in the local treatment of UM, half of patients
develop metastases typically to the liver with poor survival: 13 months
in our large retrospective series of 470 unselected metastatic patients.
Microscopic complete (R0) surgical resection of liver metastases improves
survival to 22 months in high selected patients. Early identification highrisk
patients might allow early detection of metastases, and increase R0
liver surgery rate.
Methods. From October 2006 to December 2009, we conducted a
prospective study to detect early minimal lesions by liver MRI in
asymptomatic high risk patients. High-risk was defined by thickness
>8 mm or diameter >15 mm, or extrascleral extension, or monosomy
3 for enucleated patients. Primary objective was to increase R0 liver
resection rate from 10 to 30% (α risk=0.04 and β risk=0.05); secondary
objectives were overall survival, metastasis-free survival, predictive
value of MRI and liver functional tests (LFT’s).
Results. 100 eligible patients were enrolled: median age 59 (32-83), sex
ratio M 47/F 53. The median largest tumour diameter was 18 mm (11-
26), median tumoral thickness was 11.7 mm (2.7-17); retinal detachment
was present in 75 patients, extraocular spread in 8.
Local treatment of the primary tumour consisted in proton beam therapy
in 10 patients, enucleation in 90. Secondary enucleation was performed
in 3 patients (2 for local relapse). The histotype was epithelioid in 20
cases, spindle-cell in 21, mixed in 49. Monosomy 3 (FISH or array-CGH)
was present in 55/86 (64%) analysed enucleations.
With a median follow up of 32 months, the median metastasis-free
survival was 31 months. Of 50 patients who became metastatic, 41 (82%)
had exclusive liver metastasis. 5/21 operated patients had R0 liver
surgery (24%). To date, 27 patients died (26 from metastasis, 1 myocardial
infarction) and 24 patients are alive with metastasis. 3-year overall survival
is 64%, median not reached. Univariate analysis showed gender, histotype
and monosomy 3 to be prognostic factors for metastasis.
For 85 patients with a complete follow-up dataset, 39 (46%) developed
metastasis. LFT’s failed to detect metastasis before the first abnormal
MRI; despite sensitivity and specificity rates of 100% and 83% for
nodular lesions, liver MRI failed to detect capsular miliary in 7/13
patients with peroperative confirmed liver miliary disease.
Conclusions. MRI and LFT’s screening did not increase R0 liver surgery
rate in 100 high risk patients; further studies will focus on molecular
screening for detection of circulating tumour-derived DNA in sera from
high-risk UM patients.
Financial disclosure. None
3 UM16
PERSONALIZED TARGETED THERAPY FOR UVEAL
MELANOMAS HARBORING GNAQ OR GNA11 MUTA-
TIONS
Vasiliki Poulaki1, Sue Anne Chew2, Bin He2, Nicholas Mitsiades2
(poulakiv@gmail.com)
1. Department of Ophthalmology, Veterans Affairs Boston Healthcare
System, Jamaica Plain/Boston, MA 02130; and School of Medicine,
Boston University, Boston 2. Departments of Medicine and Molecular
and Cellular Biology, Baylor College of Medicine, Houston,USA
Purpose. Inhibitors of B-Raf and MEK kinases hold promise for treatment
of cutaneous melanomas harboring BRAF mutations. BRAF mutations are
rare in uveal melanomas (UMs), but somatic mutations in the G protein
α subunits Gαq and Gα11 (encoded by GNAQ and GNA11, respectively),
occur, in a mutually exclusive pattern, in ~80% of UMs.
Methods. Using cell viability, proliferation and apoptosis assays, and
UVEAL MELANOMA
Abstracts
101
immunoblotting, we assessed the impact of the B-Raf inhibitor PLX4720,
the MEK inhibitor AZD6244, the Akt inhibitor MK2206 and the protein
kinase C (PKC) inhibitors bisindolylmaleimide I (GF109203X), Gö6983
and rottlerin on UM cell lines.
Results. BRAF–mutant UM cells were sensitive to both PLX4720 and
AZD6244, undergoing cell cycle arrest. The effect of these agents
was enhanced when administered together or in combination with
MK2206. UM cells with a Gα–protein mutation (GNAQ or GNA11) were
mildly sensitive to AZD6244, but completely resistant to PLX4720 (even
when administered in combination with AZD6244 or MK2206). In fact,
PLX4720 paradoxically increased ERK phosphorylation in Gα–mutant
UM cells. Conversely, Gα–mutant UM cells were more sensitive to all
three PKC inhibitors than BRAF–mutant UM cells.
Conclusions. The response of UM cells to inhibition of B-Raf, MEK, Akt
and PKC is genotype-dependent. Our observation that Gα–mutant UM
cells are sensitive to PKC inhibitors supports the design of future clinical
trials of such targeted therapies for the treatment of carefully selected
UM patients and provides the rationale for personalized therapy for this
highly lethal malignancy.
Financial disclosure. None
132 UM17
RATIONALE, STUDY DESIGN AND ACCRUAL STATUS
OF A RANDOMIZED PHASE II STUDY OF AZD6244, A
MEK INHIBITOR, VS TEMOZOMIDE IN ADVANCED UVEAL
MELANOMA
Richard D. Carvajal, Jedd D. Wolchok, Paul B. Chapman, Mark Dickson,
Mark Bluth, Grazia Ambrosini, Brian Marr, Murk Heinemann, Annie
Fusco, Marta Nalysnyk, Natasha Martin, Austin Doyle, Boris Bastian,
David Abramson, Gary K. Schwartz (carvajar@mskcc.org)
All from Memorial Sloan-Kettering Cancer Centre, New York, New York
except for Austin Doyle who is from the Investigational Drug Branch,
Cancer Therapy Evaluation Program, Bethesda, Maryland
Purpose. Gain of function Gnaq/11 mutations are found in 80% of uveal
melanomas (UM; vanRaamsdonk et al, Nature 2009/NEJM 2010) leading
to MAPK activation. We demonstrated growth suppression associated
with PERK and cyclinD1 down-regulation in Gnaq/11 mutant UM cell lines
with MEK inhibition (MEKi; Ambrosini et al, AACR 2010). To rigorously
assess clinical efficacy of MEKi, we are conducting a randomized
multicenter phase II trial of AZD6244 (potent MEK1/2 inhibitor) versus
temozolomide in advanced UM.
Methods. 120 DTIC/temozolomide naïve patients will be randomized
to AZD6244 75 mg PO BID or temozolomide 150 mg/m2 daily x 5 (28
days cycles), with crossover to AZD6244 permitted. Stratification factors
include Gnaq/11 mutation status. Up to 39 DTIC/temozolomide exposed
patients may receive AZD6244 without randomization. Radiographic
response is measured using RECIST 1.1, with exploratory fluoro-Lthymidine
PET studies performed in 10 patients treated with AZD6244.
Matched tumour biopsies will be collected from 30 patients receiving
AZD6244 for in vivo pharmacodynamic assessment.
Results. In the first year of accrual, 39 patients have provided informed
consented from 4 participating centres, with 25 initiating study therapy.
Another 14 centers are in the process of activating the trial. Baseline
and post-AZD6244 FLT-PET scans have been performed in 8 cases, with
paired tumour biopsies collected from 15 patients.
Conclusions. MEKi is a rationale investigational therapeutic strategy
in UM, with Gnaq/11 status a potential predictor of sensitivity. Despite

the rarity of UM, timely completion of this large trial with embedded
correlative studies appears feasible in the multicenter setting.
Financial disclosure. None
2212 UM18
A CLINICAL PROBE FOR TRANSSCLERAL OPTICAL
SPECTROSCOPY OF INTRAOCULAR TUMOURS
J Krohn1,2, P Svenmarker3, CT Xu3, S Andersson-Engels3 (joerkroh@
online.no)
1. Department of Clinical Medicine, Section of Ophthalmology, University
of Bergen, Bergen, Norway
2. Department of Ophthalmology, Haukeland University Hospital,
Bergen, Norway
3. Department of Physics, Lund University, Lund, Sweden
Purpose
To develop a fiber optic probe for in vivo diagnosis of intraocular
tumours by means of diffuse reflectance spectroscopy, and to optimize
its geometric parameters for transmitting and receiving light through
the sclera.
Methods
Two probe parameters were investigated: the source-detector distance
and the fiber protrusion, i.e. length of the fibers extending from the probe
end. Fluorescence stained choroidal tumour phantoms in enucleated
porcine eyes were measured with diffuse reflectance- and laser-induced
fluorescence spectroscopy. To evaluate the amount of light entering the
tumour phantom, rather than merely the scleral tissue, the fluorescence
from the phantoms was compared with the transmitted excitation light.
The IOP and tissue temperature were monitored, and the scleral surface
was imaged by scanning electron microscopy.
Results. A fiber source-detector distance of 5 mm with 0 mm fiber
protrusion provided maximum contrast of light interacting with the
tumour phantom relative to light propagating between the fibers without
entering into the phantom volume. Scleral applanation by the probe led
to a mean IOP rise of 15 mm Hg. During spectroscopy, a temperature rise
of 0.3 ºC was measured between the sclera and the tumour phantom.
The indentation of the optical fibers did not cause any visible damage
to the sclera.
Conclusions. A source-detector distance of 5 mm with 0.5 mm fiber
protrusion was considered optimal in terms of clinical and spectroscopic
parameters. The study indicates that transscleral spectroscopy can be
safely performed in human eyes, without leading to unacceptable IOP
elevation, significant temperature rise, or scleral damage.
Financial disclosure. Supported by grants from the Western Norway Regional Health Authority
300 UM19
OUR EXPERIENCE WITH TRANSPUPILLARY THERMO
THERAPY FOR SUSPECTED OR SMALL CHOROIDAL
MELANOMAS
Mordechai Rosner, Iris Moroz , Josef Moisseiev, Victoria Vishnevskia-Dai
(mrosner@post.tau.ac.il)
Goldschleger Eye Institute, Sackler Faculty of Medicine, Tel-Aviv
University, Sheba Medical Center, Tel-Hashomer, Israel
Purpose. Changes in treatment approaches to choroidal melanomas
resulted in better preservation of the eyeball and the visual acuity,
UVEAL MELANOMA
Abstracts
102
without increasing the mortality. As it is assumed that the initial spread
of metastases is during the proliferating stage, it might be vital to
diagnose and treat choroidal melanomas during the very early stages.
In this presentation we summarize our experience with treatment of
posterior choroidal nevi suspected for malignant transformation or small
choroidal melanomas, less than 2.5 mm in thickness using Transpupillary
Thermo Therapy (TTT).
Methods. Six patients affected by small posterior choroidal melanoma
were treated with TTT as a sole treatment from 2007 to 2011, and are
followed up.
Results. Six patients affected by small posterior choroidal melanoma
were treated with TTT as a sole treatment from 2007 to 2011, and are
followed up.
Conclusions. TTT might be the treatment of choice for selected, very
small melanomas. However, studies with long follow-up and large
number of patients are needed to evaluate it effectiveness.
Financial disclosure. None
1842 UM20
RESECTION OF IRIS TUMOURS : INTERNAL APPROACH
Arun D. Singh M.D. (singha@ccf.org)
Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic
Foundation, Cleveland, OH, USA
Purpose. To describe a novel minimally invasive surgical technique for
resection of small iris tumours.
Methods. The surgical technique is described as “Small Incision Internal
Resection and Aspiration.” Through a 3.0 mm beveled clear corneal
incision a viscoelastic is instilled into the anterior chamber. Using 25 gauge
horizontal vitrectomy scissors and vitrectomy forceps, the lesion is excised
en bloc with a visible tumour free margin. A segment of clear plastic tubing
(diameter 3.5 mm), primed with viscoelastic, is inserted into the anterior
chamber through the enlarged corneal incision. Controlled aspiration
allows removal of the entire lesion into the tube. The tube is then withdrawn
from the anterior chamber and the lesion expressed onto filter paper. The
iris defect is closed using a modified Siepser slip-knot technique. The larger
corneal incision is closed with interrupted 10-0 nylon sutures.
Results. This technique has been performed on 6 patients with localized
iris tumours (base size : 5.0-1.7 mm) (hemangioma [1] and melanoma
[5]). Reduced postoperative morbidity (inflammation, hypotony,
astigmatism) allowed rapid uneventful recovery in all cases (Va 20/20).
The histopathologic diagnosis could be established in each case and the
margins were negative in all cases.
Conclusions. Minimally invasive technique for resection of selected cases
of iris tumours avoids the potential morbidity associated with a large
corneoscleral incision allowing for rapid visual recovery.
Financial disclosure. None
1917 UM21
PLAQUE RADIOTHERAPY TREATMENT WITH RUTHE-
NIUM-106 FOR IRIS MALIGNANT MELANOMA
Amit K. Arora, Maria Tsimpida, Victoria M.L. Cohen, John L. Hungerford
(amitkarora@hotmail.com)
St. Bartholomew’s Hospital and Moorfields Eye Hospital, London
Purpose. To report results of Ruthenium-106 plaque radiotherapy for iris
malignant melanoma

Methods. A retrospective study of 15 patients with pure iris melanoma
treated with Ruthenium-106 plaque radiotherapy from June 1998 to June
2006. The main outcome measures were tumour control and ocular
complications.
Results. Of the 15 patients, 8 had biopsy-proven melanoma (6 incisional
and 2 excisional biopsies). In the remaining 7, patients enlargement
of the lesion was documented. The median follow-up was 96 months
(ranging from 14 months to 12 years). Common radiation-related
complications included cataract in 9 (60%) patients, dry eyes in 3(20%)
patients and elevated intraocular pressure in 4 (27%) patients. Vision
was preserved in 80% of patients. Local tumour control was obtained
in all patients.
Conclusions. Ruthenium-106 plaque radiotherapy is an effective
treatment for primary malignant iris melanoma , resulting in excellent
local control with preservation of vision. Main complications included
cataract ,dry eyes and glaucoma.
Financial disclosure. None
1849 UM22
RUTHENIUM BRACHYTHERAPY WITH THE 10 MM
PLAQUE FOR SMALL POSTERIOR CHOROIDAL MELA-
NOMAS
Tero Kivelä, Susanna Salkola, Sebastian Eskelin, Jorma Heikkonen
(tero.kivela@helsinki.fi)
Department of Ophthalmology and Oncology, Helsinki University Central
Hospital, Helsinki, Finland
Purpose. To assess tumour control and morbidity after brachytherapy
with 10 mm ruthenium plaques for small posterior choroidal melanomas.
Methods. In 1998-2009, forty-five consecutive choroidal melanomas were
scheduled for irradiation with the 10 mm plaque. The median height and
LBD were 1.9 (range, 0.4-5.2) and 7.0 (range, 3.3-9.6) mm, respectively.
All tumours were T1aN0M0, Stage I (7th edition; 11 were T2, 6th edition).
Median distance was 3.0 mm (range, 0-7.5) from disk and 2.0 mm (range,
0-8.5) from foveola. The anterior margin was posterior to the equator for
all except one. Median dose was 116 Gy (range, 80-194) to apex and 327
Gy (range, 201-824) to base.
Results. Four marginal recurrences developed a median of 1.4 years
(range, 0.6-3.0) after irradiation; two were also vertical (apical and basal
dose was 126-129 Gy and 266-324 Gy, respectively; median distance
from disk 1.4 mm; range, 0.4-3.8). Kaplan-Meier recurrence rate was 10%
(95% CI, 4-24) by 5 years. No patient developed signs of metastases. Six
patients, one with a prior recurrence, died a median of 4.2 years (range,
0.28-8.6) after treatment; 5-year all-cause mortality was 12% (95% CI
5-29). Regarding complications by 5 years, 50% (95% CI, 32-66) were
free of any maculopathy and 97% (95% CI, 80-100) were free or optic
neuropathy. Mean visual acuity was 0.8 at diagnosis and 0.63 at 5 years,
based on logMAR.
Conclusions. The results compare favourably with an recurrence rate of
0.25 by 5 years after transpupillary thermotherapy for tumours of similar
size and location.
Financial disclosure. None
63 UM23
REGRESSION OF UVEAL MELANOMA FOLLOWING
IODINE 125 PLAQUE RADIOTHERAPY: PREDICTIVE
FACTORS AND CORRELATION WITH METASTASIS
UVEAL MELANOMA
Abstracts
103
Hakan Demirci, Fiorella Saponara, Adam Khan, Leslie Niziol,Grant
Comer,David Musch (hdemirci@med.umich.edu)
W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI
Purpose. To analysis uveal melanoma regression following iodine 125
plaque radiotherapy, and evaluate its predictive factors and correlation
with metastasis.
Methods. Retrospective analysis of 124 eyes with > 3 years follow-up
Results. By 50% increase in follow-up time, decrease in tumor thickness
was 0.99 mm, 0.57 mm and 0.3 mm for tumors with initial thickness >
8 mm, 3-8 mm and

C. Metz, T. Gkika, W. Sauerwein, N. Bornfeld (claudia.metz@uk-essen.de)
1. University Hosital of Essen, Department of Ophthalmology, Essen,
Germany
2. University Hospital of Essen, Department of Radiation Oncology,
Essen, Germany
Purpose. Aim of this retrospective study is to analyze results of
vitreoretinal surgery following a complicated course after plaque
brachytherapy. Main focus of interest is the latency period between
plaque brachytherapy and vitreoretinal surgery and the role of
applied irradiation dosage.
Methods. We present a retrospective analysis of vitreoretinal
surgeries following brachytherapy of malignant uvea melanoma with
Ruthenium, Iodine or Ruthenium/Iodine plaques between 1991 and
2011.
Results. A total number of 172 pars-plana-vitrectomies (126 patients)
could be analyzed. Major indications for vitrectomy were massive
vitreous bleeding (53%), retinal detachment (27%) and macular
pucker (5%).
Silicone oil tamponade was necessary in 27% of the cases. Average
sclera irradiation dose was 950 Gy. The time period for irradiation
retinopathy to develop in terms of rubeosis iridis, vitreous bleeding,
intraretinal bleeding and proliferations was averagely 434 days. We
observed an amaurosis in 24 cases; 16 of those had been treated
with Ruthenium/ Iodine or CCB plaques and six cases were observed
in juxtapapillary tumours. Better functional results could be achieved
in those eyes that needed to be treated due to retinal detachment
than in eyes with vitreous bleeding.
Conclusions. Frequent controls are necessary after plaque
brachytherapy not only for tumour control, but also for early
recognition of secondary complications like irradiation retinopathy
and retinal detachment. In many cases of complicated retinal
detachment preservation of eyes and their function could be achieved
applying a temporary silicone oil tamponade.
Financial disclosure. None
2230 UM 26
LONG-TERM RESULTS AFTER ENDORESECTION OF
LARGE UVEAL MELANOMAS WITH PRETREATMENT
BY SINGLE-DOSE STEREOTACTIC IRRADIATION AND
ADJUVANT BRACHYTHERAPY
E. Biewald, H. Lautner, M. Freistühler, M. Gök, W. Sauerwein, GA
Horstmann, N. Bornfeld (eva.biewald@web.de)
1.University Hospital of Essen, Department of Ophthalmology, Essen,
Germany
2.Gamma Knife Centre, Krefeld, Germany
3.University Hospital of Essen, Department of Radiation Oncology,
Essen, Germany
Purpose. The aim of this non-comparative, consecutive case series is
to evaluate the long-term results after endoresection of large uveal
melanomas in combination with pre-treatment with stereotactic gamma
knife radiosurgery and adjuvant ruthenium brachytherapy in most
cases.
Methods. Between April 1999 and April 2010, 219 patients with large
uveal melanomas underwent stereotactic radiosurgery followed by
UVEAL MELANOMA
Abstracts
104
endoresection of the tumour via a standard three-port vitrectomy
including laser photocoagulation and silicone oil tamponade. 153
patients were treated with adjuvant ruthenium brachytherapy. Patients
with a juxtapapillary melanoma or an only eye usually did not receive
a plaque. The average tumour height was 9.3 mm. The minimum dose
delivered to the tumour volume was 25 Gy.
Results. The median follow-up time was 34 months, with a range from
10 years to 66 days. 48 patients showed a ciliary body involvement.
Only 28 eyes (12.8%) with a mean tumour distance to the fovea of 4
mm went blind after 15 months on average. All in all 24 (10.95%) eyes
were enucleated due to serious complications such as pain, phthitical
globe or loss to tumour control. In 12 of these eyes the histopathological
report found vital toumor cells. After 3,7 years on average 39 (17.8%)
patients developed liver metastases. Local tumour recurrences were
observed in 12 cases leading to an additional treatment with ruthenium
or a transpupillary thermotherapy.
Conclusions. Eyes with large uveal melanomas can be salvaged by
stereotactic radiotherapy followed by endoresection. A fair residual
visual acuity was maintained in serveral patients.
Financial disclosure. None
1447 UM27
TREATMENT OF JUXTAPAPILLARY CHOROIDAL MELA-
NOMA
Maria Tsimpida, John Hungerford, Victoria M.L. Cohen (tsimpidam@yahoo.co.uk)
The Ocular Oncology Service St. Bartholomew’s and Moorfield’s Eye
Hospitals
Purpose. To compare the efficacy of proton beam radiotherapy (PBRT)
and Ruthenium-106 notched plaque radiotherapy with or without
adjuvant transpupillary thermotherapy (TTT) for the treatment of
juxtapapillary choroidal melanoma.
Methods. Retrospective case notes analysis of all juxtapapillary
choroidal melanomas treated in London from May 2005 to February
2011. Three treatment groups were identified: group 1 PBRT; group
2 notched plaque with adjuvant TTT; group 3 notched plaque. Data
analysis was based on tumour dimensions, length of follow-up, tumour
control, radiation related complications, loss of 2 or more lines of Snellen
visual acuity and secondary enucleation rates.
Results. All juxtapapillary melanomas were ≤2mm from the optic disc.
The tumour dimensions were similar in all 3 groups. 93 melanomas were
identified. 52 with more than a year follow-up were included in the study.
Tumour control was 100% in the proton beam group, 91% in the notched
plaque with TTT group and 80% in the notched plaque group. However
radiation-related complications were much more severe in the proton
beam group, as 91% of patients lost 2 or more lines of vision, compared to
43% of patients in the notched plaque group. The secondary enucleation
rate of 9% was the same in all 3 groups.
Conclusions. Juxtapapillary melanomas can be successfully treated using
either proton beam or notched plaque combined with adjuvant TTT. However,
vision is often sacrificed. Notched plaque alone provides reduced local tumour
control but results in improved visual outcome.( word count: 245)
Financial disclosure. None
2307 UM28
OPTIC NERVE DAMAGE AFTER IRRADIATION OF
INTRAOCULAR TUMOURS

Evangelos S. Gragoudas, Anne Marie Lane, Ivana Kim
(evangelos_gragoudas@meei.harvard.edu)
Retina Service, Massachusetts Eye and Ear Infirmary, Harvard Medical
School, Boston, MA
Purpose. To evaluate the tolerance of the intraocular optic nerve to
different doses of proton irradiation.
Methods. We evaluated 1121 patients with choroidal melanoma treated
with protons in whom the optic nerve was exposed to doses between
3.5 Gy and 70 Gy. The relationship between radiation exposure and
incidence of radiation papillopathy and optic atrophy (disc pallor and
visual acuity of NLP) was assessed. Vision loss (worse than 20/200)
due to radiation-induced damage to the optic nerve was evaluated
in a subgroup of 171 patients with tumours 46.7 Gy).
Conclusions. These data suggest that exposure of the optic nerve
to radiation doses up to 47 Gy is well-tolerated, with visual loss only
observed in patients who received higher doses (> 47 Gy).
Financial disclosure. None
133 UM29
PROTON BEAM RADIOTHERAPY OF PARAPAPILLARY
CHOROIDAL MELANOMA
G.D. Willerding1, D. Cordini2, N.E. Bechrakis4, M.H. Foerster5, J. Heufelder2,
A.M. Joussen1, N. Lakotka1, L. Moser3(gregor.willerding@charite.de)
1. Charité, BerlinProtonen am HZB, Berlin, Germany; 2. Charité,
Augenklinik, Campus 1. Charité, Augenklinik, Campus Benjamin Franklin,
Berlin, Germany; 2 Charité, BerlinProtonen am HZB, Berlin, Germany; 3
Charité, Klinik für Strahlentherapie, Campus Benjamin Franklin, Berlin,
Germany; 4. Department of Ophthalmology, Medical University of
Innsbruck, Innsbruck, Austria; 5.Department of Ophthalmology, DRK
Kliniken Berlin - Westend, Berlin, Germany
Purpose. To report the results of optic disc involvement in proton beam
radiotherapy of posterior choroidal melanoma.
Methods. Retrospective case series of patients without endoresection
following radiation, where treatment planning has been done with
EYEPLAN. 159 patients, who received a cumulative dose of 50-60
CGE to the optic disc during fractionated proton beam irradiation of
choroidal melanoma between December 1998 and December 2005 were
identified.
Results. Radiation treatment controlled the tumour in 94 %. Medium
follow-up was 64 (4 to 140) months. Mean visual acuity before
radiotherapy was 0,3 and dropped to 0.05 and 0.04 after 3 and 5 years.
Mean tumour thickness decreased from 3.7 to 2.5 and 2.1 after 3 and 5
years. Recurrences occured in 6%. Main complications were radiation
optic neuropathy in 81% and vitreous bleeding in 30% irrespective of
secondary treatment. Secondary enucleation was performed in 3%.
UVEAL MELANOMA
Abstracts
105
Metastasis developed in 15% during follow-up.
Conclusions. Significant decrease in visual acuity develops after optic
disc irradiation in proton beam therapy. Rates of tumour control and eye
retention in this group of patients are favourable.
Financial disclosure. None
1414 UM30
HEMODYNAMIC MODIFICATIONS OF THE RETINA
AND THE CHOROÏD FOLLOWING PROTON BEAM
IRRADIATION OF UVEAL MELANOMAS
Leonidas Zografos, Ann Schalenbourg, Line Chamot, (leonidas.
zografos@fa2.ch)
Jules-Gonin Eye Hospital, Lausanne, Switzerland
Purpose. To study the mechanisms of hemodynamic modifications of
the retina and the choroïd following proton beam irradiation of uveal
melanomas.
Methods. Panoramic ICG and panoramic fluorescein angiography was
performed with the Staurenghi contact lens and the HRA2 camera.
223cases of uveal melanomas, previously treated with proton beam
irradiation. The main hemodynamic modifications of the choroïd and
retina are: Partial choroïdal Ischemia 58%, vortex vein occlusion 49%,
arterio venous communications between the posterior cilliary arteries
and the vortex veins 29 %, localised retinal ischemia 54 %, diffuse
retinal ischemia 18%, inferior retinal ischemia 22%.
Results. Following laser coagulation a decrease of the vascular
density of the choroïd was observed in all the cases associated to a
decrease of the permeability of the previously observed arterio venous
communications.
Conclusions. Panoramic ICG and fluorescein angiography provide useful
informations about the hemodynamic modifications of the retina and the
choroïd following proton beam irradiation of uveal melanomas as well
as after extensive laser coagulation witch allows to introduce valuable
antivasoprolifératives strategies.
Financial disclosure. None
2358 UM31
PERIOCULAR TRIAMCINOLONE FOR PREVENTION OF
MACULAR EDEMA FOLLOWING PLAQUE RADIOTHER-
APY OF UVEAL MELANOMA: THREE YEAR FOLLOW-
UP
Noel Horgan, Carol L. Shields, Melissa Murphy, Arman Mashayekhi,
Pedro F. Salazar, Miguel A. Materin, Myra O’Regan, Jerry A. Shields
(noelhorg@hotmail.com)
Royal Victoria Eye and Ear Hospital, Dublin
Wills Eye Institute, Philadelphia
Trinity College, Dublin
Purpose. To determine the efficacy and safety of periocular triamcinolone
acetonide (40 mg) for the prevention of macular edema in patients
undergoing plaque radiotherapy for uveal melanoma.
Methods. A prospective randomized controlled clinical trial recruited nehundred
and sixty-three patients with newly diagnosed uveal melanoma
undergoing Iodine125 plaque radiotherapy . Fifty-five patients were
randomized to the control group and 108 to the triamcinolone group.

Patients in the triamcinolone group received periocular injection of
triamcinolone acetonide (40 mg in 1 ml) at the time of plaque radiotherapy
and 4-months and 8-months later. Optical coherence tomography (OCT)
was performed at each patient evaluation.
Results. OCT-evident macular edema occurred significantly less often in
the triamcinolone group compared to the control group up to 36-months
following plaque radiotherapy (relative risk 0.61, 95% CI 0.46-0.81). At
36-month follow-up however, rates of moderate vision loss (loss of 3 or
more lines of best corrected visual acuity [BCVA]) and severe vision loss
(BCVA 10 mm in LBD) were included in this
prospective study. Patients were scheduled for I-125 brachytherapy
and randomized for adjuvant IVTA (4mg/0.1mL) or IVA (1.25mg/0.05mL)
injection performed at plaque removal. All patients underwent a 1, 3, 6
month follow-up examination, and every 6 months thereafter, including
fundus photography and A/B scan ultrasonography. Follow-up was
longer than 24 months.
Results. Follow-up was 37±7 months. Tumour regression was
achieved in all patients. Complete SRD regression was documented in
eighteen IVTA- treated patients (56%) vs eleven IVA-treated patients
(34%) (p

control group received both an intravenous and oral placebo. Patients
in both groups had access to standard medications for pain on a
sliding scale as needed based on pain severity. Nurses were blinded
to the patient’s group status. Patients were asked to score their pain
experience using a visual analogue scale (VAS) three times per day
during the inpatient admission while the plaque was in place as well as
once the day after plaque removal and once at the 2-week postoperative
visit. The nurse administering the steroid or placebo to the patient
separately scored his/her perception of the patients’ pain using the
Wong-Baker FACES scale.
Results. Preliminary results indicate that there is a decreased pain in
the patients in the steroid group both during plaque application and
in the immediate post-operative period. Patient recruitment and data
analysis is ongoing and full results will be presented at the ISOO.
Conclusions. The use of intravenous and oral steroids should be
considered both during the treatment period and post-operatively for
plaque patients given the severe discomfort that many plaque patients
experience.
Financial disclosure. None
1623 UM35
UVEAL MELANOMA AMONG FINNISH CHILDREN AND
ADOLESCENTS
Rana’a Aljamal, Tero Kivelä (ranaa.aljamal@hus.fi)
Departement of Ophthalmology, Ocular Oncology Service, Helsinki
University Central Hospital,
Purpose. To report uveal melanoma (UM) before the age of 25 years in
Finland, a high incidence region for this cancer.
Methods. A population-based study identified 24 patients (0.3%), aged
between 13 and 24 years at diagnosis, treated in our hospital in 1963-
2006. They were divided in two groups, the first consisted of 11 patients
(9 females, 2 males) enucleated before 1997. The second was treated by
irradiation after 1991, consisting of 11 patients (9 females, 2 males). Two
other patients underwent local resection (male and female).
Results. Tumour height was 4-11 mm (mean, 7) in the first and 4.4-13.7
mm (mean, 8.5) in the second group and largest basal diameter ranged
from 5-16 mm (mean, 10) and 2.5- 21 mm (mean, 15), respectively. Four
patients died, 3 of UM (after 4, 12 and 21 years). All were female with
spindle tumours. In the second group, 2 females died of UM (after 3
and 4 years).
Conclusions. UM is rarely seen among young children and adolescents.
In this small series, females outnumbered males.
Financial disclosure. None
UVEAL MELANOMA
Abstracts
107
406 UM 36
RESULTS OF MELANOMA BRACHYTHERAPY IN AR-
GENTINA
Arturo Irarrazaval, Pablo Cazon (artuira@consultoresoftalmologicos.
com)
Consultores Oftalmologicos
Purpose. To present our experience in 58 cases of choroidal melanoma
treated with brachytherapy in Argentina.
Methods. Retrospective study of the charts of 58 patients treated with
brachytherapy.
Results. There were, till today, 3 cases of metastases. We had 3
recurrences, and could preserve the globe in 52 cases. 31 of our patients
had a final visual acuity of 20/400 or better.
Conclusions. Conservative treatment of melanoma, using brachytherapy,
has good results in preserving the eye, without increase in mortality,
and in more than half the patients, retaining useful vision.
Financial disclosure. None

1350 Ump101
INDUCED EXPRESSION OF PIRES-EGR1-EGFP-OMI/
HTRA2 AND ITS EFFECT ON APOPTOSIS OF HUMAN
CHOROIDAL MELANOMA OCM-1 CELLS
Xufang Sun, Tian Yu, Shu Yan (applesxf@yahoo.com.cn)
Department of Ophthalmology, Tongji Hospital, Tongji Medical College,
Huazhong University of Science and Technology; Wuhan Aier Eye
Hospital, Wuhan, China
Purpose. The hydrogen peroxide induced and the kill characteristics of
pIRES-Egr1-EGFP-Omi/HtrA2 in human choroidal melanoma OCM-1 cells
were investigated.
Methods. pIRES-Egr1-EGFP and pIRES-Egr1-EGFP-Omi/HtrA2
recombinant plasmids were constructed and RT-PCR assays were
used to identify, which were transfected into the cultured OCM-1 cells
by lipofectamineTM2000. The cells transfected by both plasmids
were stimulated respectively by hydrogen peroxide. pIRES-Egr1-EGFP
transfected cells were used as control groups. The expression levels of
Omi/HtrA2 and XIAP were examined by Western blotting. The rations of
apoptotic cells were assessed by flow cytometry. The transfection rates
was observed by fluorescence microscopy. MTT method was used for
observing cell proliferation.
Results. Our Results showed that the recombinant plasmids could
successfully constructed and transferred into OCM-1 cells by
lipofectamineTM2000. Compared with the control group, the Omi/
HtrA2 and XIAP gene expression at the protein level were decreased
53.5% and 31.9%, respectively. The apoptotic ratio of pIRES-Egr1-EGFP-
Omi/HtrA2 transferred cells increased significantly after the induction
(p

1949 Ump104
GENDER DIFFERENCES AND ESTROGEN AND PRO-
GESTERONE RECEPTOR EXPRESSION IN UVEAL MEL-
ANOMA
L. Schoenfield, M. Turell, P. Carver, S. Mackie, R. Tubbs, A. Singh
(schoenl@ccf.org)
Cleveland Clinic, Cleveland, OH; Pathology and Lab Medicine Institute; Cole
Eye Institute
Purpose. Older reports have not demonstrated estrogen receptor
(ER) expression in uveal melanoma (UVM). A recent study reporting
differences in incidence and metastasis-related mortality by gender
prompted a re-examination of ER and progesterone receptor (PR)
expression by immunohistochemistry with comparison to clinical
outcome and chromosome 3 status.
Methods. 33 cases of UVM from 2004-09 treated by enucleation were
examined for incidence and survival. 23 cases were evaluated for ER and
PR by immunohistochemistry. Chromosome 3 status was determined by
FISH and SNP array analysis.
Results. There were 19 men (58%) and 14 women (42%). 11 patients
were DOD: 8 men (67%) and 3 women (25%); 1 woman was alive with
metastasis. 10 men (56%) and 8 women (44%) were alive without
metastasis. 3 patients died of other causes.
ER was positive in 7 women (11 positive cases) and negative in UVM
arising in men (8 of 12 negative cases). PR was negative in 20 of 23 cases
and weakly positive or negative in 3. ER did not predict either clinical
outcome or chromosome 3 status. Of the ER positive cases, 4 were DOD
and 6 were alive without metastasis. Of the ER negative cases, 5 were
DOD and 6 alive without metastasis. When compared to chromosome
3 abnormalities, ER was positive in 9/18 monosomy 3 cases and 2/5
disomy cases.
Conclusions. Gender may play a role in UVM. ER expression was present
in 48% of cases of uveal melanoma and more likely in women in this
small study, but it does not appear to be prognostic. We propose further
study, including quantitative evaluation using image analysis.
Financial disclosure. None
2320 Ump105
DOES INTERNAL BLOOD FLOW VELOCITY CORRELATE
WITH KNOWN PROGNOSTIC FACTORS FOR PRIMARY
UVEAL TRACT MELANOMA?
Amit Arora, Vasilios P. Papastefanou, Mandeep S Sagoo, Marie Restori,
Marianne Graham, Victoria ML Cohen (amitkarora@hotmail.com)
Ocular Oncology Service, St Bartholomew’s Hospital & Moorfields Eye
Hospital, London
Institute of Ophthalmology, University College London
Cytogenetics Department, Barts and the London NHS Trust
Purpose. To examine the association of internal blood flow velocity in
primary uveal melanoma with tumour size, chromosomes 3/8 status and
histopathology type.
Methods. Between 2009 and 2011, all patients with large uveal tract
melanoma scheduled for enucleation underwent internal blood flow
velocity studies using B-mode Doppler ultrasound scans at the time of
diagnosis. Tumour height and diameters were clinically determined.
Corresponding areas and volumes were calculated for each tumour.
UVEAL MELANOMA
Posters
109
Punch biopsy tissue samples were obtained at the time of surgery
for cytogenetic analysis for the status of chromosomes 3 and 8 with
interphase FISH analysis. Cell type was determined by histopathology.
Spearman correlation coefficient was used to examine the association
of internal blood flow velocity with tumour dimensions.
Results. Twenty-three enucleation specimens underwent preoperative
blood flow velocity studies. Internal blood flow was not positively
correlated with tumour height (r=0.061, p=0.786), area (r=-0.1.7,
p=0.636) or volume (r=0.015, p=0.946). Similarly, no significant
difference was noted in regards to cell type (12.25 ± 4.99 cm/sec for
epithelioid cells, 16.1± 10.22 cm/sec for mixed cell and 11.7± 6.9 cm/sec
for spindle cell tumours respectively [mean ± 1SD] or chromosome 3/8 status.
Conclusions. Internal blood flow of primary uveal melanoma does not
appear to be associated with uveal melanoma cell type or chromosome
status. No positive correlation was found in relation to tumour
dimensions.
Financial disclosure. None
1425 Ump106
EVALUATION OF IRIS AND CILIARY BODY LESIONS
WITH ANTERIOR SEGMENT OPTICAL COHERENCE
TOMOGRAPHY (AS-OCT) VERSUS ULTRASOUND B
SCAN
M. S. Sagoo1,2,3, V. P. Papastefanou2,3, S. Hau2, S. Shah2, M.
Tsimpida2,3, V.M. Cohen2,3 (mandeep.sagoo@moorfields.nhs.uk)
1. UCL Institute of Ophthalmology
2. Moorfields Eye Hospital, London
3. Ocular Oncology Service, Barts and the London NHS Trust, London
Purpose. To compare anterior segment optical coherence tomography (AS-
OCT) versus ultrasound B scan for imaging iris and ciliary body lesions.
Methods. We prospectively evaluated 66 eyes with AS-OCT and
ultrasound B scan. Comparison of images between the two imaging
modalities was made.
Results. Thirty seven eyes (56%) had iris naevus, 13 (20%) iris pigment
epithelial cyst, 6 (9%) iris melanoma, 2 (3%) iris haemangioma, 7 (10%)
other iris lesions, and 1 (2%) ciliary body melanoma. Image analysis
revealed (AS-OCT vs ultrasound B scan) the ability to detect lesion
(62/66 (94%) vs 46/66 (70%), complete visualisation of the lesion in
26/62 (42%) vs 46/46 (100%), and posterior lesion shadowing in 51/62
(82%) vs 0/46 (0%).
Conclusions. AS-OCT offers better detection and precise measurement
but is less able to provide complete visualisation of iris lesions compared
with ultrasound.
Financial disclosure. None
114 Ump107
Β-RADIATION BRACHYTHERAPY FOR LARGE
CHOROIDAL MELANOMA: DO WE NEED A HIGH DOSE
TO THE APEX?
M. Naseripour, K.H. Ghasemi Falavarjani, R. Jaberi, A.R. Irani, Z. Azma
(masoodnp@yahoo.com)
Eye Research Center, Rassoul Akram Hospital, Tehran University of Medical
Sciences

Purpose. To report the Results of Ru-106 plaque radiotherapy for large (
>7mm in thickness) choroidal melanoma.
Methods. In this prospective interventional case series 23 patients
with a diagnosis of large choroidal melanoma with a tumour thickness
of more than 7 mm were studied. The off label use of Ru-106 plaque
therapy for these tumour size and the safety concerns were discussed
with the patients and inform consents were obtained. The estimated
dose to the tumour apex was 100 Gy and maximum permitted sclera
dose was considered 1450 Gy.
Results. Twenty-three patients including 11 men and 12 women with a
mean age of 55.6±13.7 years were treated. Patients were followed for a
mean of 29.9±22.5 months. The mean apex dose was 70.7±15.5(40-100)
Gy. Preoperative tumour thickness of 8±0.5 mm decreased to 4.1±2.1
mm at last visit (P

Oncology Department, The S. Fyodorov Eye Microsurgery Complex,
Moscow, Russia
Purpose. To compare the outcomes of combined treatment of
choroidal melanoma with Ru-brachytherapy (BT) simultaneously with
transpupillary thermotherapy (TTT) and treatment with BT alone.
Methods. Two matched groups of patients, one treated with BT and
simultaneous TTT (group BT+TTT, n=63), the other treated with BT alone
(group BT, n=70) were analyzed retrospectively. The main outcome
measures were rate of tumour regression, recurrences, enucleations,
metastases, recurrence-free and overall survival rate, assessed by
Kaplan-Meier analysis.
Results. Patients were matched according to mean age (P=0.22), mean
tumour thickness (6.4 vs 6.25mm, range 2.5-10.8mm, P=0.59), and
mean length of follow-up (42 vs 34.4 months, range 3-109, P=0.052).
Tumour largest basal diameter (13.0 vs 12.9mm), tumour location, and
mean radiation dose (apical 135 vs 136 Gy, scleral 1294 vs 1438 Gy) were
also similar in both groups (P>0.1). Treatment with BT+TTT resulted in
higher rate of tumour regression (63% vs 49% respectively, P=0.036),
lower 5-year tumour recurrence rate (96% vs 83%, P

1547 Ump113
LONG-TERM OBSERVATIONS IN SMALL, POSTERIOR
LOCATED MALIGNANT MELANOMAS OF THE CHOROID
TREATED WITH TRANSPUPILLARY THERMOTHERAPY
(TTT)
B.M. Stoffelns, K. Schöpfer (bernhard.stoffelns@unimedizin-mainz.de)
Department of Ophthalmology, Universal Medical Center of Johannes
Gutenberg - University, Mainz, Germany,
Purpose. To evaluate the long-term Results of transpupillary
thermotherapy (TTT) for small malignant choroidal melanomas.
Methods. In the time period 1.1998 to 10.1999 in a prospective nonrandomized
analysis 26 eyes with small malignant melanomas (located
posterior to the equator with base ≤ 12 and thickness ≤ 4,5 mm) were
primary treated with the TTT standard protocol (follow-up over a time
span of 10 years minimally).
Results. Thirteen women and thirteen men (mean age 64 years)
underwent TTT. The mean preoperative tumor thickness was 2,45 mm
(0,8 – 4,5 mm). Ten years postoperatively tumor regression without
recurrence after 1,4 treatment sessions (mean) was achieved in 16/26
eyes, primary regression followed by tumor regrowth in 6/26 eyes and
primary failure of tumor regression in 4/26 eyes. Two patients died on
liver metastasis. Ocular complications (with preference in posterior
tumors after multiple TTT sessions) were observed in 14 eyes: macular
pucker in 8, macular edema in 6, choroidal neovascularisation in 4 and
posterior synechia with iris atrophy in one eye.
Conclusions. Choroidal melanomas treated with TTT as stand-alone
procedure need a close monitoring since these tumors developed a
significant rate of local recurrences and ocular side-effects in the long
run.
Financial disclosure. None
602 Ump114
CANCER RISKS FOR PATIENTS WITH MYOTONIC
DYSTROPHY
Jose Pulido1, Aung Ko Win2, Promilla Perattur3, Christine Pulido3,
Noralane Lindor3 pulido.jose@mayo.edu
1. Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota,
USA; 2. Centre for Molecular, Environmental, Genetic and Analytic
Epidemiology, The University of Melbourne, Parkville, Victoria, Australia;
3. Department of Medical Genetics, Mayo Clinic, Rochester, Minnesota,
USA.
Purpose. Myotonic dystrophy (DM) types 1 and 2 are clinically similar
autosomal dominant disorders and mainly characterized by myotonia,
muscle weakness and early-onset cataracts. Several studies have
suggested there may be increased risks for neoplasms associated with
DM. We wished to quantify the risk
Methods. A cohort of 307 DM patients identified from medical records
from Mayo Clinic Rochester was retrospectively analyzed. We estimated
standardized incidence ratios of specific cancers for DM patients
compared with age- and sex-specific cancer incidences of the general
population. Age-dependent cumulative risks were calculated using the
Kaplan–Meier method.
Results. A total of 54 cancers was observed at a median age at diagnosis
of 55 years. DM patients were at an increased risk of thyroid cancer
UVEAL MELANOMA
Posters
112
(SIR 5.54, 95% CI 1.80-12.93, p=0.001), and choroidal melanoma (SIR
27.54, 95% CI 3.34-99.49, p3 Snellen visual acuity lines. OCT scans indicated
improvement in retinal thickness over the lesion and in central
retinal thickness in all treated cases. Two cases (one untreated and
one treated with anti-VEGF) presented with outer retinal tubulation.
There was no evidence of malignant transformation during follow-up.
Conclusions. Anti-VEGF treatment was effective in achieving stable
visual acuity (loss of

1617 Ump116
INCIDENCE OF METASTATIC DISEASE AND
SURVIVAL OF 716 CONSECUTIVE PATIENTS WITH
POSTERIOR UVEAL MELANOMA : A RETROSPECTIVE
MONOCENTRIC REVIEW
Fatima Hammouch1, Patrick De Potter2, David Francart3, François
Dall’Armellina4, Jean-François Baurain5 (fatima.hammouch@uclouvain.be)
1. Oncology Department, Centre du Cancer, Cliniques Universitaires
Saint-Luc, Université Catholique de Louvain ; 2. Ophtalmology
Department, Centre du Cancer, Cliniques Universitaires Saint-Luc; 3.
Ophtalmology Department, Centre du Cancer, Cliniques Universitaires
Saint-Luc ; 4. Oncology Department, Centre du Cancer, Cliniques
Universitaires Saint-Luc, Université Catholique de Louvain ; 5.
Oncology Department, Centre du Cancer, Cliniques Universitaires
Saint-Luc, Université Catholique de Louvain, Brussels.
Purpose. To determine the rate, time and location of systemic
metastases in patients with posterior uveal melanoma and to evaluate
risk factors and survival after diagnosis of systemic relapse.
Methods. A retrospective review was performed on 716 consecutive
patients, referred and managed at the Centre du Cancer, Brussels,
for posterior uveal melanoma, between January 1998 and December
2010. Data were analyzed with SPSS 18 for Mac. The rate of metastatic
disease was calculated and patients survival was assessed by Kaplan-
Meier method. The impact of metastatic diesase–related symptoms
on survival was analyzed by the Log-Rank test. A multivariate Cox
regression was performed to identify risk factors for metastatic
disease.
Results. The median age at diagnosis was 63 years. According to the
COMS classification, there were 43 (6%) small tumors, 379 (53%)
medium tumors, 232 (32%) large tumors and 62 (9%) tumors were
unclassified. The treatment consisted in enucleation, radiotherapy
+/- thermotherapy and thermotherapy alone in 24%, 66% and 10% of
patients, respectively. The median time between tumor diagnosis and
treatment was 9 days. The median time of follow up for the 716 patients
was 3 years. Among them, 100 patients (14%) developed metastatic
disease (71% exclusively liver localisation, 19% extraliver and liver
localisations and 5% only extraliver localisation) with a median time
to relapse of 32 months. The relapse free survival from diagnosis at 5
and 10 years was 81% and 75%, respectively. Risk factors for systemic
metastases (multivariate analysis) were: large tumor (p=0.035),
epitheloid cell melanoma (p=0.002) and later age at diagnosis
(p=0.002). The median follow-up from relapse was 34 months. Fourty
patients (40%) died from their melanoma with a median survival time
of 17 months. No difference in survival was observed between those
patients with metastasis-related symptoms and those without (p>
0.05).
Conclusions. Even with a relatively short follow-up time (36 months),
our study showed a lower rate (14%) of metastatic disease with a
higher median survival time than those previously reported. Our
cohort seemed to be homogeneous, with classical risk factors
identified. While the issue of regular systemic screening on patient
survival was not assessed, we did not find a significant difference
between asymptomatic and systemic metastasis-related symptomatic
patients. The potential impact of the treatment(s) on prognosis among
our patients with metastatic disease is under investigation.
Financial disclosure. None
UVEAL MELANOMA
Posters
113
25 Ump117
LONG LASTING SURVIVAL OF UVEAL MELANOMA
WITH EXTRAOCULAR EXTENSION
Ignacio Zeolite, Carlos Zeolite, Juan Oscar Croxatto (izeolite@yahoo.
com)
Oftar Mendoza, Universidad de Mendoza
Purpose. To report a long term survival (over 60 months) of a patient
with malignant melanoma with extraocular extension post diagnostic
FNAB with no concomitant brachytherapy.
Methods. A 80-year old female patient with malignant melanoma with
extraocular extension post FNAB with no coadyuvant brachitherapy was
treated with excisional biopsy of subconjunctival dark clumps diagnosed
as extraocular extension of uveal melanoma. She refused any choice of
treatment and was followed in a regular basis for 41 months.
Results. Survival after FNAB with the diagnosis of malignant uveal
melanoma was 51 months (46 months with extraocular extension). In this
case we present an unusual pattern of extraoclar extension as multiple
subconjunctival clumps with realatively long term stability while the
general health status of the patient was well. At the time of the declining
of the general health status the tumors grew fast and the patient died in
2 months with liver metastases. Enucleation was performed 50 months
after FNAB because of ocular infection and tumor necrosis. No other
choice of treatment was authorized for the patient nor the family.
Conclusions. Malignant melanoma of the choroid can spread
extraocularly after FNAB.
Co-adyuvant brachitherapy is indicated after FNAB. Different patterns of
extraocular extension can be observed.
Long-term stability of the lesion can be associeted with good general
health status.
Financial disclosure. None
2133 Ump118
CLINICOPATHOLOGIC CORRELATIONS OF PLAQUE
BRACHYTHERAPY FAILURE IN THE TREATMENT OF
CHOROIDAL MELANOMA
Jill R. Wells, Chris S. Bergstrom, Qing Zhang, Hans E. Grossniklaus
(jrwells14@hotmail.com)
Emory Eye Center, Oncology and Pathology Service
Purpose. To correlate the histopathologic findings in the enucleated
eyes of three patients who failed treatment for choroidal melanoma with
plaque brachytherapy.
Methods. Three patients who had undergone enucleation for failure
of plaque brachytherapy for choroidal melanoma were identified.
The clinical and histopathologic features of the enucleated eyes were
reviewed as well as immunohistochemical staining.
Results. Based on the clinicopathologic correlations, case 1 failed
secondary to inadequate radiation to the peripheral tumor; case 2 failed
secondary to inadequate radiation to the tumor over the nerve; and case
3 failed secondary to inherent radioresistance of the tumor.
Conclusions. Careful attention to tumor ultrasound measurements and
correct plaque design is important. Tumors overhanging the optic nerve
are the difficult to treat. Further studies are needed to determine if and
which cellular and genetic factors in choroidal melanomas underlie
radioresistant cellular phenotypes.
Financial disclosure. None

2038 Ump119
VALUE OF DOPPLER ANALYSIS IN THE REGRESSION
OF UVEAL MELANOMA AFTER PLAQUE
Mónica Asencio-Duran, Pilar Garcia-Raya, Pilar Moreno, Isabel
Rodriguez-Rodriguez, Eva Corredoira (masedur@hotmail.com)
Hospital La Paz, Madrid, Spain
Purpose. To study the vascularization of melanoma as a sign of tumoral
activity.
Methods. 50 cases of melanoma treated with brachytherapy from
July 2005 to June 2010 were reviewed. The median follow-up was 29
months (13.7-69 months). Doppler was performed at diagnosis and
every 6 months. The average age was 60 years; there were 26 men and
24 women. 70 % of tumors were melanotic, 18 % amelanotic and 12
% mixed. Posterior location 30 %, equatorial 22 %, peripheral 20 %,
ciliary body 12 % and postequatorial 16 %. The median basal size at
diagnosis was 12.1 mm and median thickness 5.6 mm. The most used
plaque was the COMS (70 %), Ruthenium (28 %) and in 10 cases TTT
was associated. The apical dose was 85 cGy in all.
Results. Doppler detected intratumoral vascularization at diagnosis in
21 of 50 cases (42 %), of which 7 persisted at 6, 12 and 18 months. At
24 months this was 5 of 31 (16 %), at 30 months 3/20 (15 %), at 36
months 1/12 (8 %), at 42 months 1/6 (16.7 %), at 48 months 1/3 (33.3
%), at 54 months 1 of 2 (50 %) and at 60 months 0/1 (0 %). 8 avascular
tumors at diagnosis experienced new vascularization, of them, only 1
recurred and was enucleated, and 7 presented with ophthalmoscopic
and echographic regression. Of those with persistent vascularization
during the follow-up (6), 1 case recurred and was enucleated, another
developed metastasis, and 4 are regressed melanomas.
Conclusions
Results suggest that doppler is an important tool in management
of melanoma after plaque. Persistent intratumoral vascularization
seems to be associated with large tumour sizes, tumoral recurrence or
appearance of vascular congestion in neovascular glaucoma. The new
vascularized cases can be explained by the persistence of old vessels
not found before (wrong angulation of the probe), tumour recurrence or NVG.
Financial disclosure. None
65 Ump120
EVALUATION OF CHOROIDAL TUMORS BY OCT-
ENHANCED DEPTH IMAGING
Hakan Demirci, Dolly A. Padovani-Claudio, Alexis Smith, Brandon Smith,
Grant M. Comer (hdemirci@med.umich.edu)
W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI
Purpose. To evaluate the role of OCT-enhanced imaging technique (OCT-
EDI) in the imaging of choroidal tumors.
Methods. Prospective, single-center case series of 30 patients
Results. Hyporeflectivity was seen in choroidal nevus and melanoma,
while hyper-reflectivity was seen in treated choroidal melanoma,
choroidal metastasis, circumscribed choroidal hemangioma, posterior
scleritis, and choroidal hemorrhage. OCT-EDI successfully imaged the
tumors up to 1.5 mm in thickness.
Conclusions. OCT-EDI is helpful in imaging of choroidal tumors up to 1.5 mm
in thickness. It might be used in the differential diagnosis and follow-up of
these tumors.
Financial disclosure. None
114
1608 Ump121
HISTOPATHOLOGIC FINDINGS IN EYES WITH
CHOROIDAL MELANOMA TREATED WITH
BEVACIZUMAB FOR RADIATION RETINOPATHY
Hans E. Grossniklaus1, Martina C. Herwig1, 2, Weiqing Gao1 (ophtheg@
emory.edu)
1. Emory University School of Medicine, Atlanta, Georgia USA and 2.
University of Bonn, Bonn, Germany
Purpose. To describe the histopathological findings in two choroidal
melanoma eyes treated with intravitreal bevacizumab for radiation
retinopathy due to brachytherapy.
Methods. The clinical course of two patients with choroidal
melanoma who received intravitreal bevacizumab injections for
radiation retinopathy following brachytherapy was evaluated. After
enucleation for tumor recurrence (case 1) and secondary glaucoma
(case 2), the eyes were routinely processed, stained with H&E and
PAS, and immunostained for HMB45, VEGF, Ki67, CD31, and CD105.
Results. An increased amount of fibrosis within the tumor and peritumoral
tissue was found in both eyes in addition to the degenerative
changes of radiation retinopathy. HMB45 and VEGF were expressed
in both uveal melanomas, the latter particularly in macrophages.
Proliferative activity measured by the Ki67 index and angiogenesis
indicated by CD105+ vascular channels were related to tumor activity.
Conclusions. Bevacizumab injections for radiation retinopathy
seem to expedite fibrotic changes within uveal melanoma and the
peri-tumoral tissue including the choroid and the overlying retina.
Detection of tumor recurrence may be impaired by these bevacizumab
induced changes leading to a delay until adequate treatment.
Financial disclosure. None
64 Ump122
TREATMENT OF RADIATION MACULOPATHY WITH
INTRAVITREAL INJECTION OF BEVACIZUMAB
Mosci Carlo, Francesca Nasciuti, Francesco Baldo Lanza
(carlo.mosci@galliera.it)
Ocular Oncology Center, Galliera Hospital, Genova, Italy
Purpose. To evaluate the safety and efficacy of intravitreal injection
of bevacizumab as a treatment option for radiation maculopathy
secondary to proton beam radiotherapy.
Methods. Prospective case series of five patients affected by
intraocular melanoma who developed radiation maculopathy after
treatment with proton beam.
Three intravitreal injections of bevacizumab (0.5 ml) were given,
with an interval of one month between injections. The main outcome
measures were visual acuity measured with ETDRS acuity chart,
macular function with microperimetry test and optical coherence
tomography.
Results. The pre-injection visual acuity ranged from 46 to 13 with an
average of 33 letters.
The pre-injection central macular thickness measured by optical
coherence tomography ranged from 321 m to 605 m with an average
of 445 m. Three months after the first injection, the average visual
acuity was 35 and the average thickness 418; after 6 months these
numbers were 39 and 387, respectively. Microperimetry confirmed
the improvement in visual function at six months.

Conclusions. In this series, treatment of radiation maculopathy with
intravitreal injection of bevacizumab was useful in all the five patients;
macular function and macular thickness were better since the second
injection and resulted in a stable situation at six month of follow-up.
Longer follow-up and a larger number of cases are necessary for definitive
results.
Financial disclosure. None
327 Ump123
EPIDEMIOLOGICAL ANALYSIS OF BRAZILIAN
PATIENTS WITH UVEAL MELANOMA SUBMITTED TO
PRIMARY ENUCLEATION IN A REFERRAL CENTER
Priscilla L. Ballalai, Kelcia Kieffer, Ricardo Filippo, Rafaello Salla, Maria
C. Martins, Márcia Lowen (pbbordon@terra.com.br)
Ocular Oncology Unit, Federal University of Sao Paulo
Pathology Unit, Federal University of Sao Paulo
Purpose. To analyze the epidemiological characteristics of patients with
uveal melanoma submitted to primary enucleation in a referral center
in Brazil.
Methods. Retrospective, non-comparative case series. Review of
charts of patients submitted to primary enucleation from 2001 to 2011.
Epidemiological data such as age, gender, race, histological type,
presence of extra ocular extension and predisposing conditions were
analyzed.
Results. Eighty-four patients were submitted to primary enucleation
from 2001 to 2011. The mean age was 48.8 years-old (range, 19-84 yo);
45 were male and 39 females. Fifty-five patients were white, 11 black
and 1 Brazilian Indian. The information about the race was not found in
17 cases. Two patients had Oculodermal melanosis as a predisposing
condition (2.3 %) and one tumor originated from a melanocytoma (1.2
%). Nine patients had tumors at the cilliary body (11 %). Regarding
the histological type, 63 (75 %) patients had mixed type tumors, with
predominancy of epithelioid cells in 19 (30%), and spindle cells in 17
(27%). Fifteen (18 %) tumors were epithelioid type and 5 were spindle
cell type (6 %) . Extra-ocular extension was found in 7 patients (8 %).
Conclusions. Although Brazilian population is multi-racial, in this group,
uveal melanoma was more frequent in middle-aged white patients, as
previously reported. The mixed type was more prevalent. Oculodermal
melanosis and melanocytoma were rarely found as predisposing factors
for uveal melanoma.
Financial disclosure. None
2252 Ump124
UVEAL MELANOMA CLINICAL TRIALS AT MD
ANDERSON CANCER CENTER
Scott E. Woodman1A, Michael Tetzlaff1B, Xiaoxing Yu1A, Chandrani
Chattopadhyay1A, Michelle Williams1B, Nancy Poindexter1A, Elizabeth
Grimm1A, Dan Gombos3, Bita Esmaeli3, Agop Bedikian1A, Sapna Patel1A
(sewmdphd@yahoo.com)
1A. Melanoma Medical Oncology;1B, Pathology, MD Anderson Cancer
Center, Houston, TX; 2. Ophthalmology, Leiden University Med Center,
Leiden, The Netherlands; 3. Head & Neck Surgery/Ophthalmology, MD
Anderson Cancer Center, Houston, TX.
Purpose. Metastatic uveal melanoma has a very poor prognosis and
115
no effective treatment. We have designed two phase II clinical trials
specifically for patients with metastatic uveal melanoma. The first
study attempts to capitalize on the observation that metastatic uveal
melanoma cells have a propensity for elevated IGF-1R levels which may
be associated with worse outcome. The second study capitalizes on the
observation that the anti-apoptotic molecule Bcl-2 may be a mediator of
resistance to melanoma cell death after cytotoxic therapy.
Methods. Study I is a pilot study of IMC-A12 in patients with metastatic
uveal melanoma. It is designed as a prospective, multi-institutional,
two-stage, Phase II trial with 18 patients in stage I and 14 patients in
stage II. IMC-A12 is a recombinant human IgG1 monoclonal antibody
which specifically targets the human IGF-1R resulting in blockade of
ligand binding and internalization and degradation of IGF-1R. IMC A-12 is
administered at 10 mg/kg by intravenous (IV) infusion over 1 hour every
two weeks.
A treatment cycle is defined as 4 weeks. Response evaluation is
performed every two cycles. Study II is a pilot study of Genasense-
Carboplatin-Paclitaxel (GCP) in patients with metastatic uveal
melanoma. It is designed as a prospective, single-institution, twostage
Phase II trial with 15 patients in each stage. Genasense is an
antisense molecule that blocks Bcl-2 function. Genasense (900 mg) is
administered on a fixed-dose basis as over 1 hour by IV infusion on days
1, 3, and 5 of each cycle. Paclitaxel 175 mg/m2 is administered in 250 mL
NS over 1 hour by IV infusion on Day 3 of each cycle immediately upon
completion of administration of Genasense. Carboplatin (AUC= 6) is
administered in150 mL D5W over 30 minutes (+/- 5 mins) by IV infusion
after paclitaxel on day 3 of each cycle. A treatment cycle is defined as 3
weeks. Response evaluation will be performed every two cycles.
Results IMC-A12 study: The primary endpoint is objective response rate.
The secondary endpoints are safety and tolerability, disease control rate,
duration of response, progression-free survival, and overall survival.
The exploratory endpoints are to correlate response to mutation status,
correlate response to IGF-1R expression, determine the effect of IMC-
A12 on expression of proteins involved in initiation, growth, and spread
of uveal melanoma cells, determine potential resistance mechanisms to
IMC-A12. GCP study: The primary endpoint is objective response rate.
The secondary endpoints are overall survival, time to progression, time
to treatment failure, duration of response, and safety profile of GCP
in this patient population. The exploratory endpoints are correlation
of response to blood and tumor markers and potential correlation of
response to mutation status.
Conclusions. We have designed two Phase II clinical trials using more
targeted therapeutic approaches in an attempt to improve outcome.
In addition, these studies have incorporated robust correlative studies
to better interrogate molecular markers that may be associated with
clinical outcome.
Financial disclosure. None
1912 Ump125
UVEAL MELANOMA: TRENDS IN INCIDENCE,
TREATMENT, AND SURVIVAL
Arun D. Singh, Mary E. Turell, Allan K. Topham (singha@ccf.org)
Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic,
Cleveland, OH and Coalition of National Cancer Cooperative Group Inc.,
Philadelphia, PA.
Purpose. To determine trends in incidence, treatment, and survival with
primary uveal melanoma in the
United States over a 36-year period from 1973 to 2008.

Methods. A total of 4070 patients with primary uveal melanoma [ICD-
O-2] codes C69.3 [choroid], C69.4 [ciliary body and iris], and C69.2
[retina] derived from the Surveillance, Epidemiology, and End Results
(SEER) database. The significance of trends were determined using chisquare
test.
Results. There were 4070 cases of uveal melanoma representing 3.1%
of all recorded cases of melanoma.
The majority of cases (98.3%) were reported by hospitals. Histopathologic
confirmation was available in 72.1% of cases. The mean age-adjusted
incidence was 5.1 per million, with 97.8% of cases occurring in the white
population. No change in the 5-year relative survival rate (81.6%) was
observed.
Conclusions. The age-adjusted incidence (5.1 per million) has remained
unchanged from 1973 to 2008. Despite a shift toward more conservative
treatments, survival has not improved during this time period.
Financial disclosure. None
453 Ump126
FISH: MAKING HEADS OR TAILS OF TECHNIQUES
M. Turell1, R. Tubbs2 , C. Biscotti3 , Y. Sun2, Y. Saunthararajah4, P.
Triozzi 5, A. Singh1 (turellm2@ccf.org)
1. Cole Eye Institute; 2. Department of Molecular Pathology; 3.
Department of Anatomic Pathology; 4. Hematologic Oncology & Blood
Disorders; 5. Solid Tumor Oncology, Cleveland Clinic Foundation,
Cleveland, OH
Purpose. Tumor monosomy 3 confers a poor prognosis in patients
with uveal melanoma. A critical review of the literature pertaining to
techniques and procedures used for fluorescence in situ hybridization
(FISH) detection of monosomy 3 was performed in order to compare
practice patterns across oncology centers worldwide.
Methods. A PubMed literature search was conducted for studies related
to FISH-based uveal melanoma prognostication published between
January 1, 1997 and January 1, 2011.
Results. A total of 29 publications were relevant to this review.
Significant variability was found in both clinical features (tumor size
and location) and the specific FISH techniques that have been used to
assess monosomy 3 status in uveal melanoma. In particular, parameters
including tissue sampling Methods (fresh tissue [n=14], fine-needle
aspiration biopsy [n=6], and paraffin-embedded tissue [n=9]), selection
of FISH probes (centromeric [n=25], locus-specific probes [n=6], whole
chromosome paints [n=2], and unspecified [n=2]), number of cells
counted (range: 40-500), and cut-point (range 5-60%) used to determine
monosomy 3 status varied widely.
Conclusions. FISH to detect monosomy 3 in uveal melanoma has not
been performed in a standardized manner. This likely affects reported
Results and limits Conclusions regarding clinical utility.
Financial disclosure. This work was supported by a Falk Trust grant and a Research to Prevent
Blindness Challenge Grant, Department of Ophthalmology, Cleveland Clinic Lerner College of
Medicine.
116

First authors
Abramson, David H.
Al-Jamal, Rana’a
Ambrosini, Grazia
Arora, Amit K.
Asencio-Duran, Mónica
Ausburger, James
Bagnatori Braga, Marcela
Ballalai, Priscilla L.
Behnam, Babak
Berry, J.L.
Bianciotto, Carlos
Biewald, Eva M.
Biscotti, C.V.
Bita Esmaeli
Blasi, Maria A.
Bonanomi, Maria Teresa B.C.
Bornfeld, Norbert
Bosaleh, Andrea
Bosscha, M.I.
Brennan, Rachel C.
Brodie, Scott E.
Bronkhorst, I.H.G.
Callejo, Sonia A.
Carvajal, Richard D.
Cebulla, Colleen M.
Chantada, Guillermo
Chévez-Barrios, Patricia
Chintagumpala, M.
Cobrinik, David
Cohen, Victoria M.L.
Colicchio, Daniel
Corrêa, Zélia M.
Croxatto, J. Oscar
Damato, Bertil E.
De Potter, Patrick
Decatur, Christina L.
Demirci, Hakan
Desjardins, Laurence
Dimaras, Helen
Donato Macedo, Carla R.
Dos Santos Soares, Juliana
Dunkel, Ira J.
Eberhart, Charles
Elizalde, Javier
Esmaeli, Bita
Fandiño, Adriana C.
Francis, Jasmine H.
Freistühler, Michael
Frenkel, Shahar
Furuta, Minoru
Gallie, Brenda L.
Gentile, Carolina M.
Ghassemi, Fariba
Giblin, Michael E.
Glasson, William
Gombos, Dan S.
Graaf, P. de
Gragoudas, Evangelos S.
Grossniklaus, Hans E.
Hadjistilianou, T.
Page/s
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91, 99
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62, 72
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32, 49
64, 81
91, 96
30, 33, 40, 55, 62, 75
29, 35
93, 104
15, 19, 33, 55, 95, 114
30, 31, 33, 42, 57
AUTHOR INDEX
118
First authors
Halaban, Ruth
Hammouch, Fatima
Harbour, J. William
Hashimoto, Camila H.
Heegaard, Steffen
Heufelder, J.
Holdt, M.
Horgan, Noel
Houston, Samuel K.
Hovland, Peter
Hungerford, John
Hurwitz, Richard L.
Irarrazaval, Arturo
Islamov, Z.
Jager, M.J.
Jhanwar, Suresh C.
Kakkassery, V.
Kaneko, Akihiro
Kashiwagi, Hiroya
Kaushik, Megha
Kenawy, Nihal
Kilic, Emine
Kim, Ivana K.
Kiratli, Hayyam
Kivelä, Tero
Kleinerman, Ruth A.
Klufas, Michael A.
Krema, Hatem
Krohn, J.
Lally, Sara E.
Laurent, Viviana
Leahey, A.
Leal, Carlos A.
Lee, Christopher Seungkyu
Lee, Sung Chul
Lee, Susan
Levy-Gabriel, Christine
Li, Helen K.
Liu, David T.L.
Lumbroso-Le Rouic, Livia
Madic, Jordan
Malbran, Enrique S.
Manquez, Maria E.
Marback, Eduardo F.
Marinkovic, M.
Marr, Brian P.
Materin, Miguel A.
Mazzini, Cinzia
McKenzie, John D
McWhae, John
Mehta, Sonul
Metz, C.
Midena, Edoardo
Moll, A.C.
Moreno-Páramo, Daniel
Mosci, Carlo
Moulin, Alexander P.
Mruthyunjaya, Prithvi
Munier, Francis L.
Murray, Timothy G.
Page/s
16
95, 113
16, 22, 64, 79, 91, 92, 100
62, 75
61, 62, 68
94, 111
33, 54
93, 105
16, 26, 93, 106
33, 56, 63, 78
30, 42
16, 26
63, 76, 78, 92, 93, 107
29, 32, 33, 35, 49, 54
91, 96
16, 22
66, 88
65, 84
65, 83
62, 73
61, 70
15
15, 21
61, 67
62, 66, 75, 87, 92, 103
31, 45
31, 43
61, 67
92, 102
64, 65, 82, 84
16, 25
29, 37
32, 50
65, 87
65, 87
34, 58
33, 54
62, 64, 72, 81
61, 67
29, 38
15, 21
91
63, 64, 65, 80, 85
62, 65, 75, 83
61, 68
31, 43
15, 16, 23, 63, 77, 92
65, 86, 92, 100
32, 33, 52, 53
63, 78
63, 79
92, 103
63, 64, 81, 92, 93, 106
32, 51
64, 81
95, 114
63, 79
62, 63, 65, 72, 78, 86
30, 43
17, 27, 31, 33, 44, 56

First authors
Naseripour, Masood
Novetsky-Friedman, D.
Ohshima, Koh-ichi
Oliver, Scott C. N.
Owusu-Agyemang, Pascal
Palioura, Sotiria
Papastefanou, Vasilios P.
Parrozzani, Raffaele
Parulekar, M.
Pe’er, Jacob
Pelayes, David E.
Piña, Yolanda
Piperno-Neumann, S.
Poulaki, Vasiliki
Pulido, Jose S.
Qaddoumi, Ibrahim
Rashid, M.M.
Reddy, M Ashwin
Rosner, Mordechai
Saakyan, S.V.
Sagoo, Mandeep S.
Sauerwein, Wolfgang
Schaiquevich, Paula
Schalenbourg, Ann
Schefler, Amy C.
Schoenfield, Lynn
Scott, Oliver
Shields, Carol L.
Shields, Jerry A.
Shigenobu Suzuki
Singh, Arun D.
Stoffelns, B.M.
Sun, Xufang
Takashi, Yamane
Teixeira, Luiz F.
Tsimpida, Maria
Tsuji, Hideki
Turell, M.E.
Ushakova, Tatiana
Vishnevskia-Dai, Vicktoria
Wei, Wenbin
Wells, Jill R
Willerding, G.D.
Wilson, David J.
Wilson, Matthew W.
Woodman, Scott E.
Xu, Xiaoliang L.
Yarovoy, A.A.
Zeolite, Ignacio
Zografos, Leonidas
Page/s
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31, 45
65, 84
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34, 58
31, 44
63, 76, 91, 95, 97, 112
91, 98
16, 23, 29, 33, 36, 58
62, 63, 73, 77
91, 97
17, 26, 34, 59
92, 100
92, 101
66, 88, 94, 112
29, 32, 37, 48
92, 103
30, 31, 32, 44, 51, 64, 81
64, 80, 92, 102
33, 54, 65, 84
63, 64, 81, 94, 109
33, 57, 61, 69, 94, 110
30, 42
64, 81
62, 63, 76, 93, 106
15, 20, 63, 78, 94, 109
64, 80
29, 30, 41, 62, 63, 65, 78, 83
33, 57, 61, 62, 63, 71, 77
31, 46
15, 18, 63, 64, 78, 91, 92, 95, 102, 115
94, 112
94, 108
33, 53
29, 32, 38, 52, 63, 77
64, 80, 92, 104
61, 65, 83
62, 66, 76, 88, 91, 95, 98, 116
29, 39
62, 73, 76
94, 108
95, 113
93, 105
15, 20, 62, 74
16, 25, 30, 40
16, 22, 95, 115
16, 25
94, 110
95, 113
93, 105
119