Introduction
Cholangiocarcinoma (CCA) is an aggressive and nearly always fatal tumor of the biliary tract.
Purpose
This review explores risk factors, epidemiology, current diagnostic approaches, and treatment of CCA arising in patients with
primary sclerosing cholangitis (PSC).
Methods
We review latest recommendations about screening strategies to enable the early detection of CCA in PSC, using CA 19-9 and
ultrasound imaging, as well as fluorescent in situ hybridization techniques to enhance the accuracy of biliary cytology. We
also review the emerging role of liver transplantation.
Keywords cholangiocarcinoma - primary sclerosing cholangitis - epidemiology - diagnosis - treatment