Systematic analysis of current outcomes of retinoblastoma from India

Abstract

Purpose/Objective: Retinoblastoma is highly curable in resource-rich nations. Outcomes are much poorer in resource-limited nations with advanced stage at presentation being a critical adverse factor. The management and outcome of retinoblastoma across India has not been previously described. Materials and Methods: Electronic databases (Pubmed, Embase, Scopus, Web of Science) were searched to identify studies published from year 2000 onwards. SIOP (2000-2012), ASCO (2000-2012) and PEDICON (2007-2012) annual meeting abstracts were hand searched. Results: Eleven studies (8 published papers, 3 scientific abstracts) with 2036 patients (range 8-1067) were selected from 829 unique studies. These included one population-based cancer registry (PBCR) report and 10 cohorts/case-series from 6 centers in 6 cities. Unilateral to bilateral disease ratio was 1-9:1. The median age of presentation was 2-3 years Males were 1.1 to 2 times more than females at different centers. Time to presentation ranged from 4.5-12 months and the disease was extraocular in 6-76% of patients. Treatment strategies were not uniform although there was some stratification by stage. Enucleation was commonly employed in intraocular retinoblastoma. The 5-year overall survival was 48% in a single population-based cancer registry report. The event-free survival for intraocular retinoblastoma was 77-100% (median 83%, 5 studies) and for regional extension retinoblastoma was 37-43% (median 41%, 3 studies) with variable follow-up. Mortality, progression/relapse rates and treatment abandonment rates were 0-57% (median 16%, 4 studies), 0-52% (median 32%, 5 studies) and 0-60% (median 32%, 7 studies) respectively. Conclusions: Outcomes of retinoblastoma overall in India are poor with advanced stage at presentation leading to more intense treatment (toxicity), loss of eye/vision, higher relapse and treatment abandonment rates. For intraocular retinoblastoma, outcomes are much better but morbidity is high as eye salvage rates are still poor.