Retinoblastoma associated with total exudative retinal detachment: Treatment and outcomes.

Abstract

To study the clinical presentation, treatment, and outcomes of eyes presenting with retinoblastoma (RB) and total retinal detachment (RD). Retrospective study of 50 eyes in 43 patients. Mean age at presentation was 15 months (median, 9 months). RB associated total RD was unilateral (n=36; 84%) or bilateral (n=7; 16%). The mean tumor basal diameter was 17 mm (median, 18 mm) and the mean tumor thickness was 13 mm (median, 12 mm). Associated features included subretinal seeds (n=26; 52%), vitreous seeds (n=13; 26%), and subretinal exudation in (n=4; 8%). Based on 8th edition of American Joint Committee on Cancer classification, the tumors were classified as cT2a (n=19; 38%), cT2b (n=28; 56%), or cT3c (n=3; 6%). Primary treatment included intravenous (n=48; 96%) or intra-arterial chemotherapy (n=2; 4%). Over a mean follow-up period of 30 months (median, 29 months; range, 7 to 62 months), RD resolved in 41 (82%) eyes, tumor recurrence was noted in 39 (78%) eyes, globe salvage was achieved in 35 (70%) eyes and one (2%) patient died due to metastasis. Relatively high tumor recurrence rate is noted in RB presenting with total RD. With appropriate treatment, 70% of the globes can be salvaged.