Retinoblastoma outcome at a single institution in South Africa

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.