Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease that is caused by progressive inflammation and fibrotic alterations of the intra-hepatic and extrahepatic bile ducts (1, 2). The inflammatory process and the formation of scar tissue cause strictures, obliterations and destructions of the biliary tree. Patients are at risk to develop cirrhosis, liver failure and malignancies. Compared to the general population, the risk for hepatobiliary malignancies in PSC has been reported to be 161- to 1560-fold increased (3, 4). This is largely because of development of cho-langiocellular carcinoma (CCA) with a lifetime prevalence of about 5-10% in PSC (5).
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