Background: The purpose of this study was to determine the survival of retinoblastoma in the United States over a 30-year period from 1975 to 2004 using a systematic review of existing databases.
Methods: Nine hundred and ninety-two cases of retinoblastoma (International Classification of Oncology [ICDO-3] codes C69.2 [retina] and C69.9 [eye, NOS]) were derived from the Surveillance, Epidemiology, and End Results (SEER) program database in the United States from 1975 to 2004. All seventeen current SEER registries were utilized to allow for optimal patient volume for statistical analysis. Survival rates were calculated by the Kaplan Meier method and differences evaluated with Log-rank and Wilcoxon tests. Cause of death was obtained and reviewed for all deceased patients. All retinoblastoma patient records were reviewed for treatments given and occurrence of second malignant neoplasms.
Results: There were a total of 990 distinct patients with retinoblastoma. Almost all cases (99.1%) were reported by the hospitals, and histopathologic confirmation was available in 87.7% of cases. Over the period of 30 years (1975-2004), the 5-year observed actuarial survival rate increased from 92.3% (1975-84) to 93.9% (1985-94) to 96.5% (1995-04). The difference in rates was statistically significant (Wilcoxon = 6.2393, p=0.0442). The proportion of cases treated with radiotherapy first increased from 20.5% in 1975-79 to 34.6% in 1985-89 and then sharply decreased to 6.5% in 2000-04.
Conclusions: Over the last 30 years, there has been gradual improvement in 5-year survival of children with retinoblastoma in the United States.