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Clinical manifestations and diagnosis of cholangiocarcinoma

Authors
Robert C Lowe, MD
Nezam H Afdhal, MD, FRCPI
Christopher D Anderson, MD, FACS
Kris V Kowdley, MD, FACP, FACG, FASGE, AGAF
Section Editor
Kenneth K Tanabe, MD
Deputy Editors
Diane MF Savarese, MD
Anne C Travis, MD, MSc, FACG, AGAF

INTRODUCTION

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".)

TERMINOLOGY

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).

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 at the point where the common bile duct lies posterior to the duodenum, distal to the insertion of the cystic duct (figure 1) [1]. Cancers arising in the perihilar region have been further classified according to the pattern of involvement of the hepatic ducts (the Bismuth-Corlette classification) (figure 2) [2,3]. Tumors involving the hepatic duct bifurcation (hilar cholangiocarcinomas) are collectively referred to as Klatskin tumors.

CLINICAL PRESENTATION

Signs and symptoms — Extrahepatic cholangiocarcinomas usually become symptomatic when the tumor obstructs the biliary drainage system. Symptoms related to biliary obstruction include jaundice, pruritus, clay-colored stools, and dark urine. Other common symptoms include abdominal pain (30 to 50 percent), weight loss (30 to 50 percent), and fever (up to 20 percent) [4-6]. The pain is generally described as a constant dull ache in the right upper quadrant. Malaise, fatigue, and night sweats may be present [6]. Cholangitis is an unusual presentation.

                                                

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Literature review current through: Nov 2016. | This topic last updated: Fri Jul 08 00:00:00 GMT+00:00 2016.
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