A malignant hepatic tumor, typically affecting the pediatric population, arising mostly in an otherwise healthy liver. The most common signs are addominal 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).

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Term information

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MedDRA:10062001

MeSH:D018197

UMLS:C0206624

ICD-10:C22.2

OMIM:114550

notation

ORPHA:449

Term relations

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