Retinoblastoma in the United States: A 40-year incidence and survival analysis

Purpose: To determine the incidence of retinoblastoma in the United States from 1973 to 2012 (40 years) and characterize the 5-year overall survival rate of the included patients. Methods: Cases of retinoblastoma were derived from the Surveillance, Epidemiology, and End Results (SEER) Program (National Cancer Institute, Rockville, MD). Incidence rates were calculated using U.S. Census Bureau data as the standard population, and trends over time were determined using the chi-square test. Hazard ratios with a 95% confidence interval (CI) were estimated for variables associated with mortality using Cox regression models. Survival rates were calculated using the Kaplan-Meier method and compared among different clinical and demographic categories. Results: A total of 879 cases of retinoblastoma were derived from the SEER databases. The annual incidence rates of retinoblastoma for a period of 40 years were 12.14 (95% CI: 11.32 to 12.96) cases per 1 million children 4 years or younger and 0.49 (95% CI: 0.36 to 0.65) cases per 1 million children between the ages of 5 and 9 years. There was no significant trend for children 4 years or younger (P = .6324) or between the ages of 5 and 9 years (P = .7695). The 5-year overall survival rates were 97.6%, 92.7%, 91.1%, and 96.4% for children diagnosed at the first, second, third, and after the third year of life, respectively (P = .0136). The 5-year overall survival rates were 92.5% for bilateral and 96.3% for unilateral cases (P = .0116). The 5-year overall survival rates were 90.8%, 92.5%, 97.6%, 97.3% for increasing time intervals (1973 to 1979, 1980 to 1989, 1990 to 1999, and 2000 to 2012, respectively; P = .0017). Conclusions: The incidence rate of retinoblastoma in the United States has remained stable for the past 40 years. Survival rate analysis indicates a significant effect of laterality of tumor, age at diagnosis, and decade of diagnosis.