title: Retinoblastoma: A SEER Dataset Evaluation for Treatment Patterns, Survival, and Second Malignant Neoplasms.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Ophthalmology, Loyola University Chicago Stritch School of Medicine, Maywood, Illinois.
authors.forename: Diana
authors.initials: D
authors.lastname: Tamboli
authors.affiliation: Coalition of Cancer Cooperative Groups, Philadelphia, Pennsylvania.
authors.forename: Alan
authors.initials: A
authors.lastname: Topham
authors.affiliation: Department of Biostatistics, Harvard School of Public Health, Boston, Massachusetts.
authors.forename: Nakul
authors.initials: N
authors.lastname: Singh
authors.affiliation: Department of Ophthalmic Oncology, Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address: singha@ccf.org.
authors.forename: Arun D
authors.initials: AD
authors.lastname: Singh
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012042:Registries; D019572:Retinal Neoplasms / Q000401:mortality / Q000628:therapy*; D012175:Retinoblastoma / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D018426:SEER Program / Q000706:statistics & numerical data*; D015996:Survival Rate / Q000639:trends; D014481:United States / Q000453:epidemiology; D055815:Young Adult