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Challenges of Cholangiocarcinoma Detection in Patients With Primary Sclerosing Cholangitis


Challenges of Cholangiocarcinoma Detection in Patients With Primary Sclerosing Cholangitis

James H Tabibian et al. J Anal Oncol.


Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease characterized by fibro-obliterative inflammation of the hepatic bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and in some cases, cholangiocarcinoma (CCA). The development of CCA in PSC is unpredictable, its surveillance and diagnosis complex, and its treatment options limited unless detected early. Herein we provide a focused review of the current literature regarding CCA surveillance in patients with PSC and discuss the diagnostic and management challenges that exist. Where evidence is limited, we present our perspective and approach as well as directions for future research.

Keywords: Neoplasm; bile duct diseases; bile duct neoplasms; biological tumor marker; cholangiography; cytological techniques; early detection of cancer; fluorescence in situ hybridization; imaging; magnetic resonance imaging; mass screening; ultrasonography.

Conflict of interest statement



Figure 1:
Figure 1:
Magnetic resonance cholangiopancreatography (MRCP) maximal intensity projection (MIP) demonstrating diffuse intrahepatic changes of primary sclerosing cholangitis (PSC) and a mid-common bile duct dominant stricture concerning (but not definitively diagnostic) for cholangiocarcinoma in PSC patient who presented with abdominal pain and rising serum bilirubin. The patient went on to have endoscopic retrograde cholangiography with biliary sampling for further evaluation of the dominant extrahepatic stricture.
Figure 2:
Figure 2:
Proposed cholangiocarcinoma surveillance algorithm for patients with primary sclerosing cholangitis. *Assumes stable findings. CA 19–9 and liver biochemistries to also be checked along with cholangiography. In addition, cytology and FISH to be checked with ERC. **Consider early subspecialist referral if worsening in serum tests or other signs or symptoms.

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