Screening and diagnosis of cholangiocarcinoma in patients with primary sclerosing cholangitis.

Overview

Primary sclerosing cholangitis (PSC) is an idiopathic cholestatic liver disease which predisposes to the development of cholangiocarcinoma (CCA). Detection of CCA in PSC patients remains difficult and CCA is often found incidentally at autopsy or in explanted livers post-transplantation for PSC. In addition, considerable overlap exists between the symptoms of CCA and those of benign dominant strictures encountered commonly in PSC. Clinicians utilize a combination of serum tumor markers, cytology from bile duct brushings, and imaging in an attempt to screen for and detect CCA in patients with PSC, although the evidence for these modalities remains largely retrospective. Newer treatment options for early CCA such as resection and liver transplantation have shown promising results, making an effective screening regimen for the detection of CCA at a treatable stage in PSC patients a highly coveted goal.