title: Cancer risk in children and adolescents with birth defects: a population-based cohort study.
abstract: OBJECTIVE
  Birth defects are an increasing health priority worldwide, and the subject of a major 2010 World Health Assembly Resolution. Excess cancer risk may be an added burden in this vulnerable group of children, but studies to date have provided inconsistent findings. This study assessed the risk for cancer in children and young adolescents with major birth defects.
  
  
  RESULTS
  This retrospective, statewide, population-based, cohort study was conducted in three US states (Utah, Arizona, Iowa). A cohort of 44,151 children and young adolescents (0 through 14 years of age) with selected major, non-chromosomal birth defects or chromosomal anomalies was compared to a reference cohort of 147,940 children without birth defects randomly sampled from each state's births and frequency matched by year of birth. The primary outcome was rate of cancer prior to age 15 years, by type of cancer and type of birth defect. The incidence of cancer was increased 2.9-fold (95% CI, 2.3 to 3.7) in children with birth defects (123 cases of cancer) compared to the reference cohort; the incidence rates were 33.8 and 11.7 per 100,000 person-years, respectively. However, the excess risk varied markedly by type of birth defect. Increased risks were seen in children with microcephaly, cleft palate, and selected eye, cardiac, and renal defects. Cancer risk was not increased with many common birth defects, including hypospadias, cleft lip with or without cleft palate, or hydrocephalus.
  
  
  CONCLUSIONS
  Children with some structural, non-chromosomal birth defects, but not others, have a moderately increased risk for childhood cancer. Information on such selective risk can promote more effective clinical evaluation, counseling, and research.
authors.affiliation: Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, Utah, USA. Lorenzo.botto@hsc.utah.edu
authors.forename: Lorenzo D
authors.initials: LD
authors.lastname: Botto
authors.forename: Timothy
authors.initials: T
authors.lastname: Flood
authors.forename: Julian
authors.initials: J
authors.lastname: Little
authors.forename: Mark N
authors.initials: MN
authors.lastname: Fluchel
authors.forename: Sergey
authors.initials: S
authors.lastname: Krikov
authors.forename: Marcia L
authors.initials: ML
authors.lastname: Feldkamp
authors.forename: Yuan
authors.initials: Y
authors.lastname: Wu
authors.forename: Rhinda
authors.initials: R
authors.lastname: Goedken
authors.forename: Soman
authors.initials: S
authors.lastname: Puzhankara
authors.forename: Paul A
authors.initials: PA
authors.lastname: Romitti
mesh_terms: D000293:Adolescent; D002648:Child; D002869:Chromosome Aberrations; D015331:Cohort Studies; D000013:Congenital Abnormalities / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D009369:Neoplasms / Q000150:complications* / Q000453:epidemiology / Q000235:genetics; D011159:Population Surveillance; D012307:Risk Factors