Clinical presentation, treatment, and outcomes of retinoblastoma in India: A literature review

Background: India has the highest incidence of retinoblastoma globally and is one of the six Asian countries identified to contribute 43% to the global retinoblastoma cases. Data on clinical presentation, treatment, and outcomes were reviewed which can serve as basis in the creation of clinical guidelines, policies and programs, and resource allocation in the diagnosis and management of retinoblastoma in India. Methodology: Articles on retinoblastoma in India gathered from different databases were reviewed for clinical features, treatment, and outcomes. Result: Fourteen articles with 3,666 patients involving around 4,945 eyes seen from 1983-2017 were reviewed. The median age at consult ranged from 14-48 months. The median delay of consult ranged from 2-9 months. Majority of the patients were males (59%) and unilateral disease was present in 63%. Family history was reported in 4%. Retinoblastoma was intraocular in at least 75% of eyes. Systemic chemotherapy was the most used treatment option given to >2,042 eyes. Enucleation was done in >1,695 eyes. The mean follow-up period ranged from 4-50 months. Three hundred fifty six (356) patients were lost to follow-up. The functional vision was retained in 134 eyes. The globe salvage rate for Group A was 100%, 94-100% for Group B, and 50100% in Group C. The highest globe salvage rate for Group D eyes was 85% and 58% for group E. The overall survival rate was 75% (2,233 patients). Conclusion: Common among the articles was the relatively high proportion of extraocular disease attributed to delay in consult attributed to financial factors and lack of knowledge on the disease.