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Disease definition

A malignant hepatic tumor, typically affecting the pediatric population, arising mostly in an otherwise healthy liver. The most common signs are abdominal distension and abdominal mass. Sometimes patients present with anorexia, weight loss, fatigue. Most HBLs are sporadic, but some cases are associated with genetic factors, especially overgrowth syndromes, such as Beckwith-Wiedemann syndrome (BWS) or hemihypertrophy, and familial adenomatous polyposis (FAP).


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Neonatal, Infancy, Childhood
  • ICD-10: C22.2
  • ICD-11: 2C12.01
  • OMIM: 114550
  • UMLS: C0206624
  • MeSH: D018197
  • GARD: 2657
  • MedDRA: 10062001

Detailed information


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