Hepatobiliary and extra-hepatic malignancies in primary sclerosing cholangitis

The increased risk for cholangiocarcinoma in primary sclerosing cholangitis (PSC) is well established, but the factors responsible for the malignant development in the bile ducts in this disease are not known. The pathogenesis of cholangiocarcinoma in PSC including the role of chronic inflammation and oncogenic mutations will be discussed. Cholangiocarcinoma is a leading cause of death in PSC and the prognosis even after liver transplantation is poor, with a median survival after cancer diagnosis of 5 months. Therefore, it is of great importance to identify PSC patients who are at risk of developing cholangiocarcinoma in order to transplant them before cancer has developed.