Background: Cholangiocarcinoma (CCA) is the most common hepatobiliary malignancy complicating primary sclerosing cholangitis (PSC). Unfortunately, timely diagnosis of CCA in PSC patients remains challenging. Aim: To investigate the strategies among liver centers regarding pre-transplant screening for CCA in patients with PSC. Methods: An online survey was returned from 46 US transplant centers, inquiring on the frequency of screening, the use of specific tests, or tactical approaches to high-grade dysplasia (HGD) or CCA. Results: Most centers screen their PSC patients for CCA prior to orthotopic liver transplantation (OLT) (89%). Serum carbohydrate antigen 19-9 and magnetic resonance cholangiopancreatography are first-line screening tools (93 and 84% respectively). Endoscopic retrograde cholangiopancreatography with biliary brushings is routinely performed in only 30% of the centers. In the case of HGD, 61% would choose close monitoring. In the event of non-resectable CCA, 37% have an OLT protocol, 33% resort to palliative treatment and the remaining 30% make an outside referral. Finally, half the participating centers perform CCA surveillance among their listed PSC patients every 6 months. Conclusion: Screening for CCA among PSC patients prior to OLT varies greatly among centers. Serum carbohydrate antigen 19-9 and magnetic resonance cholangiopancreatography are widely used. HGD warrants surveillance rather than intervention among most experts. Protocolized chemoradiation followed by OLT has yet to become a widely accepted approach. The very poor survival of PSC patients who develop CCA underlines the importance of an effective and universally accepted screening process that will aid in its earlier detection.

Keywords:
Cholangiocarcinoma, Cholangitis, Biliary dysplasia, Liver transplantation
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2014
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