Travel burden and clinical presentation of retinoblastoma: analysis of 1024 patients from 43 African countries and 518 patients from 40 European countries

BACKGROUND: The travel distance from home to a treatment centre, which may impact the stage at diagnosis, has not been investigated for retinoblastoma, the most common childhood eye cancer. We aimed to investigate the travel burden and its impact on clinical presentation in a large sample of patients with retinoblastoma from Africa and Europe. METHODS: A cross-sectional analysis including 518 treatment-naïve patients with retinoblastoma residing in 40 European countries and 1024 treatment-naïve patients with retinoblastoma residing in 43 African countries. RESULTS: Capture rate was 42.2% of expected patients from Africa and 108.8% from Europe. African patients were older (95% CI -12.4 to -5.4, p<0.001), had fewer cases of familial retinoblastoma (95% CI 2.0 to 5.3, p<0.001) and presented with more advanced disease (95% CI 6.0 to 9.8, p<0.001); 43.4% and 15.4% of Africans had extraocular retinoblastoma and distant metastasis at the time of diagnosis, respectively, compared to 2.9% and 1.0% of the Europeans. To reach a retinoblastoma centre, European patients travelled 421.8 km compared to Africans who travelled 185.7 km (p<0.001). On regression analysis, lower-national income level, African residence and older age (p<0.001), but not travel distance (p=0.19), were risk factors for advanced disease. CONCLUSIONS: Fewer than half the expected number of patients with retinoblastoma presented to African referral centres in 2017, suggesting poor awareness or other barriers to access. Despite the relatively shorter distance travelled by African patients, they presented with later-stage disease. Health education about retinoblastoma is needed for carers and health workers in Africa in order to increase capture rate and promote early referral

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

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