Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis

Cholangiocarcinoma (CCA) is the most common malignancy in primary sclerosing cholangitis (PSC). Approximately half of CCA are diagnosed within two years of initial diagnosis and often have a poor prognosis because of advanced tumor stage at the time of diagnosis. Thus, rigorous initial imaging evaluation for detecting CCA is important. CCA in PSC usually manifests as intrahepatic mass-forming or perihilar periductal-infiltrating type. Imaging diagnosis is often challenging due to pre-existing biliary strictures and heterogeneous liver. Multimodality imaging approach and careful comparison with prior images are often helpful in detecting small CCA. Ultrasound is widely used as an initial test, but has a limited ability to detect small tumors in the heterogeneous liver with PSC. MRI combined with MRCP is excellent to demonstrate focal biliary abnormalities as well as subtle liver masses. Contrast-enhanced ultrasound is useful to demonstrate CCA by demonstrating rapid and marked washout. In addition, there are other disease entities that mimic CCA including hepatocellular carcinoma, confluent hepatic fibrosis, IgG4-related sclerosing cholangitis, inflammatory mass, and focal fat deposition. In this pictorial essay, imaging findings of CCA in PSC is described and discuss the challenges in imaging surveillance for CCA in the patients with PSC. Imaging findings of the mimickers of CCA in PSC and their differentiating features are also discussed.