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Adenocarcinoma of the liver and intrahepatic biliary tract

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Disease 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.

ORPHA:424943

Classification level: Disorder

Synonym(s):
  • Adenocarcinoma of the liver and IBT

Prevalence: -

Inheritance: Not applicable

Age of onset: Adult

ICD-10: C22.0

UMLS: C5192429

A summary on this disease is available in Français (2019) Español (2019) Deutsch (2019) Italiano (2019) Nederlands (2019)
Detailed information
Guidelines
Clinical practice guidelines
English (2023) - Ann Oncol Logo ERN

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

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