Program Book

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