Program Book

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)