November 29, 2015
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Bile Duct Cancer

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What is bile duct cancer (cholangiocarcinoma)? What are causes and risk factors for bile duct cancer?

Bile duct cancer arises from the cells that line the bile ducts, the drainage system for bile produced by the liver. Bile ducts collect this bile, draining it into the gallbladder and finally into the small intestine where it aids in the digestion process. Bile duct cancer is also called cholangiocarcinoma.

Bile duct cancer is a rare form of cancer, with approximately 2,000 new cases diagnosed in the United States each year. There are three general locations where this type of cancer may arise within the bile drainage system:

  • Within the liver (intrahepatic) affecting the bile ducts located within the liver
  • Just outside of the liver (extrahepatic or perihilar) located at the notch of the liver where the bile ducts exit
  • Far outside of the liver (distal extrahepatic) near where the bile ducts enter the intestine (called the ampulla of Vater)

Bile duct cancers are most commonly found just outside of the liver in the perihilar area and least commonly found within the liver.

The incidence of bile duct cancer increases with age. It is a slow-growing cancer that invades local structures and for that reason, the diagnosis is often made late in the disease process when the bile ducts become blocked. This blockade prevents bile drainage from the liver into the gallbladder and intestine. Depending upon where the blockage occurs, this can lead to inflammation of the liver (hepatitis) and/or pancreas (pancreatitis).

Most patients who develop bile duct cancer have no risk to do so. However, chronic inflammation of the bile ducts may be a risk factor for this cancer. Diseases that can cause this type of chronic inflammation include primary sclerosing cholangitis (especially when associated with ulcerative colitis), chronic liver disease, including hepatitis B, hepatitis C, chronic alcoholic hepatitis, and cirrhosis.

Certain parasitic infections found in the Far East that cause liver infections are associated with an increased risk.

Gallstones are not a risk factor for developing bile duct cancer, but stones within the liver do pose an increased risk. Liver stones are not often seen in the North American population but are more common in Asian countries.

There are rare congenital diseases that increase the risk of bile duct cancer, including Lynch II syndrome (hereditary nonpolyposis colorectal cancer associated with biliary tree and other cancers) and Caroli's syndrome (portal hypertension, hepatic fibrosis, and biliary tree cysts).

Native Americans are six times more likely to develop bile duct cancer. Asian Americans may also be at higher risk. Bile duct cancer is also more prevalent in Israel and Japan, but it is a very rare disease in North America.

Medically Reviewed by a Doctor on 10/1/2015



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