Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Abstract

Purpose: To study epidemiology, demographic profile, clinical characteristics, and outcome in pediatric patients with retinoblastoma. Methods: This was a retrospective review of retinoblastoma patients of a tertiary institute from January 1st 1998 to December 31st 2014. Results: The study included 467 patients (618 eyes) with a mean age of 34.7 ± 24.6 months (median = 30; 15 days to 144 months). Retinoblastoma was bilateral in 151 (32.3%) and there were 61.7% males. Intraocular disease was seen in 301 patients (451 eyes [72.9%]) and extraocular in 166 patients (167 eyes; 27.0%). Out of the 347 (74.3%) who received treatment, primary treatment was chemoreduction in 228 (65.7%) and enucleation in 117 (33.7%), while 25.6% of patients refused treatment and 151 (43.5%) defaulted therapy. Local recurrence was seen in 20 (4.3%), metastasis in 2 (0.4%), and deaths in 13 (2.8%) (average follow-up 28.5 ± 44.4 months). Histopathological high risk features were significantly less in the eyes that received chemoreduction (5.0%) versus primary enucleation (20.8%) (p < 0.0004), but there was no difference in the rate of metastasis, recurrence, and death between the two. Conclusions: The majority of retinoblastoma patients in our study had advanced disease, and nearly a third had extraocular extension. There were a significant number of therapy refusals and dropouts. Chemoreduction led to a significant decrease in the histopathological risk factors without affecting the outcomes.