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Epidemiology, pathogenesis, and classification of cholangiocarcinoma

Robert C Lowe, MD
Christopher D Anderson, MD, FACS
Section Editor
Kenneth K Tanabe, MD
Deputy Editors
Diane MF Savarese, MD
Kristen M Robson, MD, MBA, FACG


Cholangiocarcinomas (bile duct cancers) arise from the epithelial cells of the intrahepatic and extrahepatic bile ducts. Although these cancers are rare in the United States, they are highly lethal because most are locally advanced at presentation.

The epidemiology, pathology, pathogenesis, and classification of cholangiocarcinoma will be discussed here. Clinical manifestations, diagnosis, and treatment are reviewed separately. (See "Clinical manifestations and diagnosis of cholangiocarcinoma" and "Treatment of localized cholangiocarcinoma: Adjuvant and neoadjuvant therapy and prognosis" and "Treatment options for locally advanced cholangiocarcinoma" and "Systemic therapy for advanced cholangiocarcinoma".)

Cancers of the gallbladder and ampulla of Vater are discussed as separate disease processes, although these structures are part of the biliary drainage system. (See "Gallbladder cancer: Epidemiology, risk factors, clinical features, and diagnosis" and "Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging".)


Biliary tract cancers were traditionally divided into cancers of the gallbladder, the extrahepatic ducts, and the ampulla of Vater, while intrahepatic tumors of the bile system were classified as primary liver cancers. More recently, the term cholangiocarcinoma has been used to refer to bile duct cancers arising in the intrahepatic, perihilar, or distal (extrahepatic) biliary tree, exclusive of the gallbladder or ampulla of Vater (figure 1).

Intrahepatic cholangiocarcinomas originate from small intrahepatic ductules (termed peripheral cholangiocarcinomas) or large intrahepatic ducts proximal to the bifurcation of the right and left hepatic ducts. The extrahepatic bile ducts are divided into perihilar (including the confluence itself) and distal segments, with the transition occurring at the point where the common bile duct lies posterior to the duodenum, proximal to the insertion of the cystic duct into the common bile duct [1].

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Literature review current through: Nov 2017. | This topic last updated: Jun 29, 2017.
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