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Clinical manifestations and diagnosis 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 bile ducts. Although they are rare in the United States, these cancers are highly lethal because most are locally advanced at presentation. (See "Epidemiology, pathogenesis, and classification of cholangiocarcinoma".)

The clinical manifestations and diagnosis of cholangiocarcinoma will be reviewed here. The epidemiology, pathogenesis, classification, and treatment of cholangiocarcinoma are discussed separately. 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 "Epidemiology, pathogenesis, and classification of cholangiocarcinoma" and "Treatment of localized cholangiocarcinoma: Adjuvant and neoadjuvant therapy and prognosis" and "Treatment options for locally advanced cholangiocarcinoma" and "Gallbladder cancer: Epidemiology, risk factors, clinical features, and diagnosis" and "Ampullary carcinoma: Epidemiology, clinical manifestations, diagnosis and staging".)


Extrahepatic biliary tract malignancies were traditionally divided into cancers of the gallbladder, the extrahepatic ducts, and the ampulla of Vater, whereas intrahepatic tumors were classified as primary liver cancers. More recently, the term cholangiocarcinoma has been used to designate bile duct cancers arising in the intrahepatic, perihilar, or distal (extrahepatic) biliary tree, exclusive of gallbladder and ampulla of Vater (figure 1) [1-3].

Approximately 5 to 10 percent of cholangiocarcinomas are intrahepatic. Intrahepatic cholangiocarcinomas can originate from either small intrahepatic ductules (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 proximal to the cystic duct (figure 1) [2]. Cancers arising in the perihilar region, which account for 60 to 70 percent of extrahepatic cholangiocarcinomas, have been further classified according to the pattern of involvement of the hepatic ducts (the Bismuth-Corlette classification) (figure 2) [4,5]. Tumors involving the hepatic duct bifurcation (hilar cholangiocarcinomas) are collectively referred to as Klatskin tumors.

Type IV tumors, defined as tumor invasion of the second order biliary radicles bilaterally, are associated with a higher rate of positive surgical margins and significantly poorer overall survival after resection compared with types I to III [6]. The most recent (2017) revision of the tumor, node, metastasis (TNM) classification, which goes into effect in the United States on January 1, 2018, no longer considers type IV tumors to represent a T4 primary tumor. Outside of the United States, the Union for International Cancer Control (UICC) has implemented the eighth edition changes as of January 1, 2017. (See 'Tumor staging' below.)

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Literature review current through: Nov 2017. | This topic last updated: Nov 30, 2016.
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