Retinoblastoma in Asia: Clinical Presentation and Treatment Outcomes in 2112 Patients from 33 Countries

Abstract

PurposeTo describe the clinical presentation and treatment outcomes of children diagnosed with retinoblastoma (RB) in the year 2017, throughout Asia. DesignMulti-national prospective study including treatment-naïve patients diagnosed with retinoblastoma in Asia during 2017 and followed up thereafter. ParticipantsA total of 2112 patients (2797 eyes) from 96 RB treatment centers in 33 Asian countries. InterventionChemotherapy, radiotherapy, enucleation, orbital exenteration Main Outcome MeasuresEnucleation and death ResultsWithin the cohort, 1021 (48%) patients were from South Asia (SA), 503 (24%) from East Asia (EA), 310 (15%) from South-East Asia (SEA), 218 (10%) from West Asia (WA) and 60 (3%) from Central Asia (CA). Mean age at presentation was 27 months (median, 23 months; range, <1 to 261 months). There were 1195 (57%) males and 917 (43%) females. The most common presenting complaints were leukocoria (72%) and strabismus (13%). Based on 8th edition American Joint Committee for Cancer, tumors were staged as cT1 (n=441; 16%), cT2 (n=951; 34%), cT3 (n=1136; 41%), cT4 (n=267; 10%), N1 (n=48; 2%), and M1 (n=129; 6%) at presentation. RB was treated with intravenous chemotherapy in 1450 (52%) eyes and 857 (31%) underwent primary enucleation. Three-year Kaplan-Meier estimates for enucleation and death were 33% and 13% for CA, 18% and 4% for EA, 27% and 15% for SA, 32% and 22% for SEA, 20% and 11% for WA (p<0.0001 and p<0.0001), respectively. ConclusionAt the conclusion of this study, there was a significant heterogeneity in treatment outcomes of RB between the regions of Asian continent. East Asia displayed better outcomes with higher rates of globe and life salvage, while South-East Asia had poorer outcomes compared to the rest of Asia.