title: Incidence of abdominal tumors in syndromic and idiopathic hemihypertrophy/isolated hemihyperplasia.
abstract: BACKGROUND
  There is a reported increased risk of intra-abdominal tumors in children with both syndromic (SH) and isolated idiopathic hemihyperplasia (IH). Recommendations for tumor surveillance have been made, although there is no consensus for frequency and duration of screening. Our objective was to review the incidence of abdominal neoplasms in our pediatric population with SH and IH.
  
  
  METHODS
  We reviewed the diagnostic criteria, imaging findings, and any associated syndrome in all patients diagnosed with hemihypertrophy over a 10-year period.
  
  
  RESULTS
  One of 10 patients with SH, a child with Beckwith-Wiedemann syndrome, developed a hepatoblastoma resulting in a 10% tumor incidence in patients with SH. Three of the 250 (1.2%) children with IH developed an abdominal neoplasm. One was diagnosed with adrenal carcinoma and the other 2 with Wilms tumor.
  
  
  CONCLUSIONS
  We found an increased incidence of abdominal tumors in both SH and IH, however, our incidence of tumors with IH is lower than earlier reported studies. On the basis of this lower 1.2% incidence, the current literature on IH and available molecular genetic testing, it is reasonable to recommend referral of these patients to a clinical geneticist to identify subgroups with a higher risk for tumor development that are more likely to benefit from routine imaging surveillance.
  
  
  METHODS
  II-Retrospective study.
authors.affiliation: Department of Radiology, Texas Scottish Rite Hospital for Children, Dallas, TX 75219, USA. molly.dempsey-robertson@tsrh.org
authors.forename: Molly
authors.initials: M
authors.lastname: Dempsey-Robertson
authors.forename: David
authors.initials: D
authors.lastname: Wilkes
authors.forename: Alec
authors.initials: A
authors.lastname: Stall
authors.forename: Patricia
authors.initials: P
authors.lastname: Bush
mesh_terms: D000008:Abdominal Neoplasms / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D001506:Beckwith-Wiedemann Syndrome; D002648:Child; D002675:Child, Preschool; D005820:Genetic Testing; D006130:Growth Disorders / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D006965:Hyperplasia / Q000453:epidemiology / Q000235:genetics; D006984:Hypertrophy / Q000453:epidemiology / Q000235:genetics; D015994:Incidence; D007223:Infant; D008403:Mass Screening / Q000379:methods*; D012189:Retrospective Studies; D012306:Risk; D013577:Syndrome