Cholangiocarcinoma in primary sclerosing cholangitis

J Gastrointest Cancer. 2009;40(1-2):19-25. doi: 10.1007/s12029-009-9085-8. Epub 2009 Aug 25.


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.

Publication types

  • Review

MeSH terms

  • Bile Duct Neoplasms* / complications
  • Bile Ducts, Intrahepatic / pathology*
  • Cholangiocarcinoma* / complications
  • Cholangitis, Sclerosing / complications*
  • Humans