Purpose: To characterize treatment patterns, overall survival (OS), and risk of second malignant tumors in patients with retinoblastoma (RB) using the SEER (Surveillance Epidemiology and End Results) dataset.
Design: Cohort study.
Methods: The SEER dataset was used to identify cases of RB using ICD-03 histology codes. Special permission was granted by the SEER administration to release chemotherapy information for this study (information that is not available in the publically available SEER dataset). Treatment of RB for patients with locoregional disease was characterized as surgical therapy, radiation therapy, chemotherapy, or any form thereof across 4 time periods from 1975 to 2010. Observed-to-expected (O/E) ratios for second malignant neoplasms were calculated with the use of age-specific incidence rates.
Results: There were 1452 cases of RB identified from 1975 to 2010, with 48% of patients being male and 30% presenting with bilateral disease. Treatment patterns over time of 1220 patients (84%) with localized disease showed an increase in chemotherapy (± any treatment) from 16.5% to 50.2% and a decrease in surgery (± any treatment) from 96.2% to 88.5% and decrease in radiation from 15.2% to 4.9% from the 1975-1979 time period to the 2000-2010 time period. The 10-year OS was 93.7%, 93.7%, 97.5%, and 97% for increasing time periods (1975-1979, 1980-1989, 1990-1999, 2000-2010, respectively; P = .029). Risk of second malignant neoplasms was highest among patients treated with radiotherapy with O/E ratio of 43 compared to 30 and 5 for chemotherapy and surgery alone, respectively.
Conclusion: Treatment trends for RB show an increase in chemotherapy utilization with a decrease in radiation therapy from 1975 to 2010. Second malignant neoplasms occurred mainly in patients treated with radiation therapy. Our series demonstrates improvement in survival in contemporary time periods, which parallels a shift in therapy toward chemotherapy with a decline in radiation therapy.
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