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Journal of Clinical Oncology, 2008 ASCO Annual Meeting Proceedings (Post-Meeting Edition).
Vol 26, No 15S (May 20 Supplement), 2008: 10051
© 2008 American Society of Clinical Oncology
Demographics, clinical profile, management and outcome of retinoblastoma
V. A. Reddy,
S. G. Honavar,
D. Shome,
K. Shah,
R. Murthy,
M. Naik and
G. K. Vemuganti
Apollo Cancer Hospital, Hyderabad, India; LV Prasad Eye Institute, Hyderabad, India
10051
Background: Retinoblastoma is the most common intraocular tumor in children. Comprehensive data on the clinical spectrum and outcome of protocol-based management in a large clinical series will help further understand the disease and plan future clinical trials. Methods: Our prospective case series included consecutive patients of retinoblastoma at a Retinoblastoma Center from January 1990 to December 2004 with 3y follow-up. Demographics, clinical features, treatment and outcome (survival, organ salvage and function salvage) were analyzed. Management comprised of focal therapy for small tumors, standard triple drug chemoreduction for larger (>4mm) tumors, high dose chemoreduction and periocular chemotherapy for vitreous seeds, and enucleation for advanced tumors. Adjuvant therapy was provided in patients with histopathologic high-risk factors for systemic metastasis. Orbital retinoblastoma was managed with a multimodal treatment protocol. Results: Of 460 patients, 66% presented <3y age. Although 41% were bilateral, family history of retinoblastoma was forthcoming in only 4%. Leucocoria (71%) was the most common symptom, followed by visual loss (12%), proptosis (7%) and squint (6%). The tumor was intraocular in 94% eyes while 6% were extraocular. Tumor grouping is shown in table. Enucleation was the most common primary treatment (236 of 451, 52%), followed by chemoreduction (26%), focal therapy (11%), external beam radiation (55) or chemotherapy alone (4%). Histopathologic risk factors predictive of metastasis were found in 55% of eyes that underwent enucleation. Chemoreduction with focal therapy resulted in eye and vision salvage in 109 of 118 (92%). Outcome (eye salvage) by tumor grouping is shown in the table. At the final follow-up, 434 (94%) children were alive and well. Conclusions: Retinoblastoma currently has excellent prognosis for survival, eye salvage and vision salvage. Advocacy for early diagnosis coupled with a better management approach for advanced retinoblastoma may optimize the outcome.
No significant financial relationships to disclose.
Abstract presentation from the 2008 ASCO Annual Meeting
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