Demographics, clinical profile, management, and outcome of retinoblastoma in a tertiary care center in southern India

20011 Background: Retinoblastoma is the most common malignant intraocular tumor in children. The estimated incidence of retinoblastoma in India is about 2,000 a year. However, there is scant data on the clinical manifestations and outcome of retinoblastoma in India. Methods: In this retrospective case series, the medical records of 460 consecutive cases of retinoblastoma from January 1990 to December 2004 were analyzed for demographics, clinical profile, treatment and outcome (survival, organ and function salvage). Results: An integrated Ocular Oncology Service was established at the treatment center in the year 2000 and a specialized Children’s Eye Cancer Center was founded in 2004. The annual referral increased from <20 a year before 2000 to 38 in 2000, and 120 in 2005. Two hundred and six (45%) patients were economically underprivileged. A majority (303, 66%) presented <3 years of age. There were 277 (60%) males. Although 188 (41%) were bilateral, family history was forthcoming in only 19 (4%). Of 648 involved eyes, 531 (82%) eyes were symptomatic. Leucocoria (71%) was the most common symptom, followed by visual loss (12%), eyelid edema or proptosis (7%), squint (6%), pain (2%), redness (2%), enlarged eyeball (2%), or shrunken eyeball (1%). The tumor was intraocular in 609 (94%) eyes while 39 (6%) were extraocular. Enucleation was the most common primary treatment for intraocular retinoblastoma (236 of 451, 52%), followed by chemoreduction coupled with focal therapy (26%), focal therapy alone (11%), external beam radiation (55) or chemotherapy alone (4%). Histopathologic risk factors predictive of metastasis were found in 152 of 277 (55%) eyes that underwent enucleation. Chemoreduction with focal therapy resulted in eye and vision salvage in 109 of 118 (92%). At the final follow-up (minimum 24 months), 434 (94%) were alive and well. Conclusions: Retinoblastoma has excellent prognosis for survival, eye salvage and vision salvage with the current management modalities. An integrated approach involving a team of a pediatric oncologist and an ocular oncologist at a comprehensive tertiary care center may augment referrals and help implement systematic treatment strategies. No significant financial relationships to disclose.