Cholangiocarcinoma in the setting of primary sclerosing cholangitis (PSC) is difficult to reveal and is often diagnosed at an advanced stage of tumour growth and spread, or accidentally at liver transplantation in end-stage PSC1"4, In some patients the cholangiocarcinoma will be revealed only at autopsy5. Clinically, biliary malignancy is often suspected when a PSC patient shows rapid, progressive liver disease with increasing bilirubin levels, weight loss and abdominal pain.. However, end-stage PSC without cholangiocarcinoma can also present with rapid disease progression, and it is not possible to distinguish clinically between end-stage PSC and PSC complicated by cholangiocarcinoma6. The prognosis of PSC patients with cholangiocarcinoma is dismal, with a median survival time of less than 6 months after cancer diagnosis7, and bile duct carcinoma is a leading cause of mortality in patients with PSC. Not only clinical but also radiological methods have failed to be specific and sensitive diagnostic tools, Therefore other markers are important for the diagnosis of bile duct carcinoma in PSC.
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