How Should Cancer Surveillance in Primary Sclerosing Cholangitis Be Performed?

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease affecting the intra- and extrahepatic bile duct system that can ultimately cause liver cirrhosis. Apart from the risk of progression to end-stage liver disease the prognosis of PSC is primarily determined by the risk to develop hepatobiliary or extrahepatic malignancies. A reasonable surveillance strategy for PSC patients must allow the detection of early cancer that will permit a potentially curative therapy.

METHODS: Current guidelines on malignancy within the context of PSC as well as the primary literature were reviewed for this article.

RESULTS: Here, we focus on a concise review of the three tumors most commonly associated with PSC: cholangiocellular carcinoma (CCA), gallbladder cancer, and colorectal carcinoma. For cancer surveillance in this patient group, endoscopy, cholangiography, cross-sectional imaging, and the use of serum tumor markers are principally available. Furthermore, for the diagnosis of CCA novel approaches were recently suggested to improve sensitivity and specificity to detect this malignancy.

CONCLUSION: We review different aspects of cancer surveillance in patients with PSC. Since prospective data on the surveillance of malignant tumors is unavailable, we discuss a rational approach on how to perform cancer surveillance in patients with PSC.