Retinoblastoma outcome at a single institution in South Africa

Abstract

Introduction. Retinoblastoma (RB) is the most common eye cancer in children. Early detection is necessary for cure. Objective. To compare stage and outcome of children with RB treated at Kalafong Hospital, Pretoria, South Africa (SA), during two time periods (1993 - 2000 and 2001 - 2008, after outreach interventions in 2000 and introduction of compulsory community service for doctors in 1998). Methods. Data collected included demography (age, gender, date of birth), stage and treatment received. The main outcome measure was disease-free survival and the study end-point was 60 months after diagnosis. Results. There were 51 patients during the time period 1993 - 2000 (group 1) and 73 during 2001 - 2008 (group 2), with median ages of 32 and 26 months, respectively (marginally significantly younger in group 2; p=0.046). In group 1, the majority (57%) presented with advanced disease (stages III and IV), with a decline in this proportion in group 2 (40%) indicating a downward but not significant trend (p=0.075). Bilateral disease was diagnosed in 22% of patients in group 1 and 33% in group 2. Overall survival was 33% and 43% for groups 1 and 2, respectively. Excluding absconding patients, event-free survival was 50% in group 1, improving to 68% in group 2 (not statistically significant; p=0.18). Fewer patients needed radiotherapy during the second period (statistically significant; p=0.04), probably because of less advanced disease. Conclusion. Poor outcome is probably a result of late diagnosis. It is important to implement a strategy that will ensure early diagnosis and optimal management of RB in SA.