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Extrahepatic Bile Duct Cancer Treatment (PDQ®)

Health Professional Version
Last Modified: 07/31/2014

General Information About Extrahepatic Bile Duct Cancer

Incidence and Mortality
Anatomy
Pathogenesis
Clinical Features
Treatment Modalities



Incidence and Mortality

Cancer arising in the extrahepatic bile duct is an uncommon disease, and is curable by surgery in fewer than 10% of all cases.[1]

Anatomy

Prognosis depends in part on the tumor’s anatomic location, which affects its resectability. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct, a resectability rate that is clearly better than for lesions that occur in more proximal sites.[2]

Enlarge
Anatomy of the extrahepatic bile duct; drawing shows the liver, right and left hepatic ducts, gallbladder, cystic duct, common hepatic duct (perihilar), common bile duct (distal), extrahepatic bile duct, small intestine, and pancreas. The inset shows the liver, bile ducts, gallbladder, pancreas, and small intestine.
Anatomy of the extrahepatic bile duct. The extrahepatic bile duct is made up of the common hepatic duct and the common bile duct.
Pathogenesis

Bile duct cancer may occur more frequently in patients with a history of primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, or infections with the fluke, Clonorchis sinensis.[3]

Clinical Features

The most common symptoms caused by bile duct cancer include:

  • Jaundice.
  • Pain.
  • Fever.
  • Pruritus.
Treatment Modalities

In most patients, the tumor cannot be completely removed by surgery and is incurable. Palliative resections or other palliative measures such as radiation therapy (e.g., brachytherapy or external-beam radiation therapy) or stenting procedures may maintain adequate biliary drainage and allow for improved survival. Many bile duct cancers are multifocal. Perineural invasion has a negative impact on survival.[4]

References
  1. Henson DE, Albores-Saavedra J, Corle D: Carcinoma of the extrahepatic bile ducts. Histologic types, stage of disease, grade, and survival rates. Cancer 70 (6): 1498-501, 1992.  [PUBMED Abstract]

  2. Stain SC, Baer HU, Dennison AR, et al.: Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 175 (6): 579-88, 1992.  [PUBMED Abstract]

  3. de Groen PC, Gores GJ, LaRusso NF, et al.: Biliary tract cancers. N Engl J Med 341 (18): 1368-78, 1999.  [PUBMED Abstract]

  4. Bhuiya MR, Nimura Y, Kamiya J, et al.: Clinicopathologic studies on perineural invasion of bile duct carcinoma. Ann Surg 215 (4): 344-9, 1992.  [PUBMED Abstract]