Retinoblastoma outcomes in Europe: a prospective analysis of 483 patients from 40 countries

Abstract

Purpose: To describe presentation, treatment and outcomes for a cohort of children presenting with retinoblastoma (Rb) throughout Europe during 2017. Methods: A prospective analysis of 483 patients diagnosed in Europe between Jan 1, 2017, and Dec 31, 2017, then treated and followed up for 3 years. Results: Unilateral cases 339/483 (70%) predominated, presenting older (mean age 26 months) than the 144 (30%) bilateral cases (mean age 12 months p < 0.0005). Only 4/477 (0.8%) children had extra-ocular Rb at presentation (mean age 53 months vs 21 months for those without p = 0.002). Children from middle income countries did not present older, but were more likely (p < 0.001) to present with late-stage disease (3-4) than high-income children (74/151 (49%) vs 108/332 (33%), RR 1.25 95%CI 1.09–1.44). For unilaterals, primary treatment was intravenous chemotherapy (IVC) in 29% and intra-arterial chemotherapy (IAC) in 20%. For bilaterals, primary treatment was IVC in 113/144 (78%) and IAC in 14/144 (10%). Overall, 58% of children underwent enucleation, 36% of which as primary treatment. Risk of enucleation was determined by stage and laterality, but not economic status. Twelve (2.5%) children died from Rb. More children (OR = 146-7 13.9–1549.4, p < 0.0005) presenting with extra-ocular tumour died (3 of 4 (75%)) than with intra-ocular tumour (9/449 (2%)) More children (OR = 29.8 3.8–232.0, p < 0.0005), from middle income countries died from Rb (11/132 (8%)) than from high income countries (1/327 (0.3%)). Conclusion: Even within a wealthy continent such as Europe, economic factors may influence survival, but not global salvage rates. The majority of children still lose an eye.