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Cholangiocarcinoma — ESEGH MCQ

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HardGI CancerCholangiocarcinomaESEGH

A 61-year-old man with an 18-year history of ulcerative colitis and established large-duct primary sclerosing cholangitis develops progressive pruritus, a 5 kg weight loss and worsening cholestatic liver biochemistry over 3 months. MRCP shows a new irregular eccentric stricture of the common hepatic duct extending to the hepatic duct confluence, with shouldering and disproportionate upstream dilatation. Multiphase CT shows subtle enhancing periductal soft tissue at this site, without a pancreatic or gallbladder mass or distant metastases. Serum IgG4 is normal. ERCP brush cytology is reported as suspicious for malignancy. What is the most likely diagnosis?

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Correct answer: A — Perihilar cholangiocarcinoma

The most likely diagnosis is perihilar cholangiocarcinoma. PSC substantially increases cholangiocarcinoma risk, and the combination of progressive cholestasis, weight loss, a new irregular shouldered stricture at the hepatic duct confluence, enhancing periductal tissue and suspicious brush cytology strongly supports malignancy. Its location defines it as perihilar cholangiocarcinoma. A benign PSC stricture remains an important mimic, but is less likely given the convergent clinical, radiological and cytological warning features. Normal IgG4 and the absence of typical pancreatic or other IgG4-related disease argue against IgG4-related sclerosing cholangitis. No gallbladder mass is present to support secondary hilar invasion. Intraductal papillary neoplasm more commonly produces an intraductal papillary mass, mucobilia or disproportionate duct ectasia rather than an infiltrative shouldered hilar stricture. The case requires specialist HPB multidisciplinary review because biliary cytology has limited sensitivity and a 'suspicious' result is not equivalent to definitive histological confirmation.

Reference: Rushbrook SM et al. British Society of Gastroenterology guidelines for the diagnosis and management of cholangiocarcinoma. Diagnostic pathway, risk-factor and endoscopic diagnosis sections. Gut 2024;73:16–46. https://www.bsg.org.uk/getmedia/eeb6686a-02b1-4e7d-9298-3547c9fcb972/British-Society-of-Gastroenterology-guidelines-for-the-diagnosis-and-management-of-cholangiocarcinoma.pdf%3Fext%3D.pdf