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Contributors: Michael W. Winter MD, Khaled Bittar MD, Nishant H. Patel MD, Desiree Rivera-Nieves MD
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Cholangiocarcinoma refers to biliary tree malignancies, primarily adenocarcinomas, of epithelial cell origin. They are typically categorized as intrahepatic, perihilar, or distal.

Cholangiocarcinomas often present insidiously; symptom onset often coincides with biliary duct obstruction leading to jaundice, pruritus, or cholangitis. Other less specific signs and symptoms include unexplained weight loss, lethargy, and anorexia.

Cirrhosis, hepatitis B, hepatitis C, primary sclerosing cholangitis, and hepatobiliary flukes are known risk factors for cholangiocarcinoma. However, most cholangiocarcinomas develop in patients with no identifiable risk factors. It is a rare childhood malignancy. Incidence is highest in Hispanic and Asian populations.

Patients with cholangiocarcinoma will often have elevations in their liver function tests (total and direct bilirubin) and liver enzymes (aspartate transaminase [AST], alanine transaminase [ALT], alkaline phosphatase), although these are nonspecific findings. Elevations in cancer antigen (CA) 19-9, a tumor marker, can be suggestive of cholangiocarcinoma but is limited by nonspecificity. Diagnosis typically requires tissue sampling via endoscopic retrograde cholangiopancreatography (ERCP) with brushing.

Overall prognosis is poor, as many patients present with advanced disease. Early detection of tumors that can be resected surgically portends the most favorable prognosis. Chemotherapy can slow disease progression but is rarely curative.

For more information, see OMIM.


C22.1 – Intrahepatic bile duct carcinoma

70179006 – Cholangiocarcinoma

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Last Reviewed: 09/27/2017
Last Updated: 09/27/2017
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Cholangiocarcinoma : Chills, Dark urine, Hepatomegaly, Jaundice, Chronic duration lasting years, Weight loss, Anorexia, Pruritus, RUQ pain, Pale feces
Imaging Studies image of Cholangiocarcinoma
MCRP demonstrates peripheral and central enhancement consistent with cholangiocarcinoma.
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