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Adenocarcinoma of the liver and intrahepatic biliary tract

ORPHA:424943· ICD-10 C22.0

Definition

A rare hepatic and biliary tract tumor characterized by a growth pattern ressembling that found in hepatocellular carcinomas and cholangiocarcinomas but presenting atypical histological and immunohistochemical features (such as trabecular, organoid, microcystic and/or blastemal-like architecture and inhibin A, cytokeratin 7 and/or cytokeratin 19 positivity) that do not allow a formal diagnosis of the more common aforementioned liver cancers. Patients may present abdominal distension and pain, a palpable abdominal mass and elevated liver enzymes.

Prevalence
1-9 / 1 000 000
Inheritance
Not applicable
Age of onset
Adult