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Adenocarcinoma of the gallbladder and extrahepatic biliary tract

ORPHA:424991· ICD-10 C23

Definition

A rare epithelial carcinoma, arising either in the gallbladder itself or from the epithelium lining the extrahepatic biliary tree, cystic duct and/or peribiliary gland, characterized by nonspecific symptoms, such as abdominal pain, jaundice and vomiting and sometimes mimicking benign biliary diseases. Chronic biliary epithelial inflammation (e.g. primary sclerosing cholangitis, cholelithiasis, choledocholithiasis, liver fluke infestation) is a major risk factor.

Prevalence
1-9 / 100 000
Inheritance
Not applicable
Age of onset
Adult