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Disease definition

A malignant hepatic tumor, typically affecting the pediatric population, characterized by anorexia, weight loss and an enlarged abdomen. This disorder is more common in patients with familial adenomatous polyposis (FAP), and can occur in patients with other pre-existing liver conditions. About 5 % of cases are associated with genetic factors, especially overgrowth syndromes, such as Beckwith-Wiedemann syndrome (BWS) or hemihypertrophy.


  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Neonatal, Infancy, Childhood
  • ICD-10: C22.2
  • OMIM: 114550
  • UMLS: C0206624
  • MeSH: D018197
  • GARD: 2657
  • MedDRA: 10062001

Detailed information


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