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Swathi Kaliki, Anamika Patel, Sadiya Iram, Vijay Anand Reddy Palkonda, Ashik Mohamed, George Ramappa; Retinoblastoma in India: Clinical presentation and outcome in 1457 patients (2074 eyes). Invest. Ophthalmol. Vis. Sci. 2017;58(8):3341.
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© ARVO (1962-2015); The Authors (2016-present)
India peaks the predicted incidence of retinoblastoma in the world with over 1400 of 8000 new cases diagnosed each year. In this report, we studied the clinical presentation, treatment, and outcome of patients with retinoblastoma (RB) in India. We performed a retrospective clinical study on 1457 patients (2074 eyes) with retinoblastoma.
All patients diagnosed with retinoblastoma and with adequate documentation of presenting signs and clinical details were included in the study. The treatment modalities and outcome (metastasis and death) were analyzed.
The mean age at presentation of RB was 29 months (median, 24 months; range; <1 to 370 months). There were 812 (56%) males and 645 (44%) females with unilateral presentation of RB in 57% (n=834) and bilateral in 43% (n=623). Family history of RB was present in 4% (n=55). The most common presenting complaints included leukocoria (n=1100; 75%), proptosis (n=91; 6%), strabismus (n=77; 5%), and red eye (n=68; 5%). Most (n=1889; 91%) tumors were intraocular in location and 185 (n=185; 9%) had extraocular tumor extension at presentation. Based on International Classification of Intraocular Retinoblastoma, the tumors were classified as group A (n=107; 6%), B (n=278; 15%), C (n=127; 7%), D (n=414; 22%), or E (n=963; 51%). Based on International Staging of Retinoblastoma, the tumors were classified as stage 0 (n=933; 45%), I (n=925; 45%), II (n=31; 2%), III (n=131; 6%), or IV (n=54; 3%). Based on Tumor Node Metastasis classification, the tumors belonged to T1 (n=385; 19%), T2 (n=541; 26%), T3 (n=963; 46%), or T4 (n=185; 9%). The most common modalities of primary treatment included systemic chemotherapy (n=1171; 60%) and enucleation (n=674; 35%). At a mean follow-up period of 44 months (median, 30 months; range, 3 to 234 months), 92% (n=1206) were alive and 108 (8%) patients died due to RB. Based on Kaplan-Meier analysis, the survival at 1, 3, 5, and 10 years was 94%, 91%, 90%, and 89% respectively.
The most common presenting signs of RB in Asian Indian population are leukocoria and proptosis. Though most patients present with advanced disease compared to western studies, with appropriate treatment, the survival rate is favorable at 92%.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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