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 is a type of cancer that arises from the cells that line the bile ducts, the drainage system for bile of the liver. Bile ducts collect the bile from the liver and allow it to drain into the small intestine. The bile within the intestine helps with food digestion. Bile duct cancer is also called cholangiocarcinoma.

Bile duct cancer is a rare form of cancer, with less than 3,000 new cases diagnosed in the United States each year. There are three general locations where this type of cancer arises within the bile drainage system:

  • Within the liver (intrahepatic): Affecting the bile ducts located within the liver
  • Just outside of the liver (extrahepatic): also called 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 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 prevents bile drainage from the liver into the 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 and chronic parasite infections. Patients with chronic liver disease, including hepatitis B, hepatitis C, chronic alcoholic hepatitis, and cirrhosis may also be at risk for developing bile duct cancer.

Gallstones are not a risk factor for developing bile duct cancer, but stones within the liver 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.

Medically Reviewed by a Doctor on 2/4/2015

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Ray Manzarek Dies of Bile Duct Cancer

Venice Beach, California, 1965. By good fortune, Ray Manzarek runs into Jim Morrison, a former UCLA classmate, and the world is blessed with the music of The Doors. The band sells 100 million albums.

Rosenheim, Germany, 2013. Fortunes turn for Mr. Manzarek, as he passes away from cholangiocarcinoma, bile duct cancer, a rare cancer that afflicts less than 4,000 people a year in the U.S.