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RETINOBLASTOMA IN INDIA: Clinical Presentation and Outcome in 1,457 Patients (2,074 Eyes)

Kaliki, Swathi, MD*; Patel, Anamika, MD*; Iram, Sadiya, MD*; Ramappa, George, BSc*; Mohamed, Ashik, PhD, MBBS, MTech; Palkonda, Vijay A., R., MD*

doi: 10.1097/IAE.0000000000001962
Original Study: PDF Only

Purpose: To study the clinical presentation, treatment, and outcome of patients with retinoblastoma (RB) in India.

Methods: Retrospective study of 1,457 patients with RB (2,074 eyes).

Results: The mean age at presentation of RB was 29 months (median, 24 months; range, <1–370 months). There were 812 (56%) men and 645 (44%) women with unilateral presentation of RB in 57% (n = 834) and bilateral in 43% (n = 623). Familial RB was present in 4% (n = 55). The most common presenting complaints included leukocoria (n = 1,100; 75%), proptosis (n = 91; 6%), strabismus (n = 77; 5%), and red eye (n = 68; 5%). Most (n = 1,889; 91%) tumors were intraocular in location, and 185 (n = 185; 9%) had extraocular tumor extension at presentation. The most common modalities of primary treatment–included systemic chemotherapy (n = 1,171; 60%) and enucleation (n = 674; 35%). At a mean follow-up period of 44 months (median, 30 months; range, 3–234 months), 92% (n = 1,206) were alive, and 108 (8%) patients died because of RB. Based on Kaplan–Meier analysis, the survival at 1, 3, 5, and 10 years was 94%, 91%, 90%, and 89%, respectively.

Conclusion: The most common presenting signs of RB in Asian Indian population are leukocoria and proptosis. With appropriate treatment, the survival rate is favorable at 92%.

In a study of 1,457 Asian Indian patients with retinoblastoma, 91% had intraocular tumors and 9% had extraocular tumor extension, and with appropriate treatment, 92% survival rate was achieved at a mean follow-up period of 44 months.

*Ocular Oncology Service, The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, India; and

Ophthalmic Biophysics, L V Prasad Eye Institute, Hyderabad, India.

Reprint requests: Swathi Kaliki, MD, The Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad 500034; e-mail: kalikiswathi@yahoo.com

Supported by The Operation Eyesight Universal Institute for Eye Cancer (S.K.), The Champalimaud Foundation (S.K.), and Hyderabad Eye Research Foundation (S.K.), Hyderabad, India. The funders had no role in the preparation, review, or approval of the article. Statistical analysis performed by A. Mohamed, Ophthalmic Biophysics, L V Prasad Eye Institute, Hyderabad, India.

None of the authors has any conflicting interests to disclose.

© 2018 by Ophthalmic Communications Society, Inc.