The Beckwith-Wiedemann syndrome phenotype and the risk of cancer

Med Pediatr Oncol. 1997 Jun;28(6):411-5. doi: 10.1002/(sici)1096-911x(199706)28:6<411::aid-mpo3>;2-j.


Beckwith-Wiedemann syndrome (BWS) comprises of a number of childhood abnormalities, often associated with one or more tumors. Thirty-eight patients were investigated to determine clinical and/or biological signs associated with a tumor presence. Our patients exhibited a higher incidence of tumor development (21%) than that previously reported, underlying the care with which such patients should be followed, when particular clinical features are observed: visceromegaly affecting three organs (liver, kidney, spleen), and also family history with sign of BWS such as macroglossia, omphalocele, hemihypertrophy, embryonic tumor), high body weight at birth (> or = +2 standard deviations and diastasis recti.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Beckwith-Wiedemann Syndrome / blood
  • Beckwith-Wiedemann Syndrome / genetics*
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Insulin-Like Growth Factor I / metabolism
  • Insulin-Like Growth Factor II / metabolism
  • Male
  • Neoplasms / blood
  • Neoplasms / genetics*
  • Odds Ratio
  • Phenotype
  • Risk


  • Insulin-Like Growth Factor I
  • Insulin-Like Growth Factor II