title: Second malignant neoplasms among children, adolescents and young adults with Wilms tumor.
abstract: BACKGROUND
  The goal of this study was to describe the incidence, characteristics, and outcomes of secondary malignant neoplasms (SMN) in survivors of Wilms tumor.
  
  
  METHODS
  Patients who were 0-20 years of age at time of primary diagnosis with Wilms tumor and reported to the Surveillance, Epidemiology, and End Results [SEER] program between 1973 and 2011 were eligible for inclusion in the cohort. We used competing risks methods to estimate the cumulative incidence of SMNs and assess contributing factors for developing SMN. We estimated standardized incidence ratios (SIR), absolute excess risk and overall survival after SMN using standard methods.
  
  
  RESULTS
  Within the SEER database, 2,851 patients were diagnosed with Wilms tumor as their first malignancy. Of these, 34 patients were reported to have a SMN. Cumulative incidence of for a secondary malignancy was 0.6% (95% confidence interval [95% CI] 0.3-1.0%) at 10 years, 1.6% (95% CI 1.0-2.3%) at 20 years, and 3.8% (95% CI 2.4-5.9%) at 30 years. Median time from primary diagnosis to SMN diagnosis was 12.5 years. SIR for SMN for survivors of Wilms tumor was 3.4 (95% CI 2.2-4.9) with an absolute excess risk of 7.6 per 10,000 persons per year. Exposure to radiation did not significantly increase risk for development of second malignancy. Overall survival for patients with SMN was 64.5% at 5 years.
  
  
  CONCLUSIONS
  Survivors of Wilms tumor are at an increased risk of SMN compared to the general population, but the added risk is relatively small compared to other pediatric cancers.
authors: Department of Pediatrics, UCSF Benioff Children's Hospital, University of California.
authors: Jean S
authors: JS
authors: Lee
authors: Department of Surgery, Division of Pediatric Surgery, UCSF Benioff Children's Hospital, University of California.
authors: Benjamin
authors: B
authors: Padilla
authors: Department of Pediatrics, UCSF Benioff Children's Hospital, University of California.
authors: Steven G
authors: SG
authors: DuBois
authors: Department of Pediatrics, UCSF Benioff Children's Hospital, University of California.
authors: Aris
authors: A
authors: Oates
authors: Department of Biostatistics, UCSF Benioff Children's Hospital, University of California.
authors: John
authors: J
authors: Boscardin
authors: Department of Pediatrics, UCSF Benioff Children's Hospital, University of California.
authors: Robert E
authors: RE
authors: Goldsby
mesh_terms: D000293:Adolescent; D000328:Adult; D002140:California / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000150:complications* / Q000473:pathology; D008297:Male; D009367:Neoplasm Staging; D016609:Neoplasms, Second Primary / Q000453:epidemiology / Q000209:etiology* / Q000401:mortality; D011379:Prognosis; D018426:SEER Program; D015996:Survival Rate; D009396:Wilms Tumor / Q000150:complications* / Q000473:pathology; D055815:Young Adult
keywords: Wilms tumor; late effects of cancer treatment; long-term survival; outcomes research; second malignant neoplasms