Epidemiological trends in 1452 cases of retinoblastoma from the Surveillance, Epidemiology, and End Results (SEER) registry.

objectiveTo assess retinoblastoma epidemiological trends in the Surveillance, Epidemiology, and End Results (SEER) registry.

methodsAll cases of retinoblastoma in the SEER database from 1973 to 2009 were identified. Kaplan-Meier survival analyses were performed for pathological grade, patient age, sex, year of diagnosis, and treatment modality. Cox proportional hazards regression assessed the impact of patient and tumour characteristics on survival.

results1452 cases of retinoblastoma were analyzed. The mean patient age at diagnosis was 1.44 years. The tumour was unilateral in 71.0% and bilateral in 29.0%. The mean follow-up was 129.1 months. Overall survival increased during the study interval. Patients with bilateral tumours were diagnosed at an earlier age (0.46 years) than patients with unilateral disease (1.77 years; p < 0.0001). Bilateral retinoblastoma (90.3% 10-year overall survival) was associated with decreased overall survival than unilateral retinoblastoma (96.1% 10-year overall survival). Bilateral retinoblastoma was also associated with an increased incidence of nonocular malignancies (7.8%) compared with unilateral retinoblastoma (1.3%; p < 0.0001). Grade 1 tumours were diagnosed at a younger age (0.94 years) than grade 3 (2.24 years) and grade 4 tumours (2.14 years; p < 0.0001). Lower grade and lower stage tumours were independently associated with increased survival. In multivariate Cox proportional hazards analysis, T stage and laterality were the only covariates that correlated with overall survival.

conclusionsThere appear to be associations between retinoblastoma tumour features such as tumour stage, pathological grade, and laterality with patient characteristics such as age at diagnosis, overall survival, and second malignancies.