Bile Duct Cancer (Cholangiocarcinoma)

  • Medical Author:
    Benjamin Wedro, MD, FACEP, FAAEM

    Dr. Ben Wedro practices emergency medicine at Gundersen Clinic, a regional trauma center in La Crosse, Wisconsin. His background includes undergraduate and medical studies at the University of Alberta, a Family Practice internship at Queen's University in Kingston, Ontario and residency training in Emergency Medicine at the University of Oklahoma Health Sciences Center.

  • Medical Editor: William C. Shiel Jr., MD, FACP, FACR
    William C. Shiel Jr., MD, FACP, FACR

    William C. Shiel Jr., MD, FACP, FACR

    Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

View Cancer 101 Slideshow Pictures

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.

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

Subscribe to MedicineNet's Cancer Report Newsletter

By clicking Submit, I agree to the MedicineNet's Terms & Conditions & Privacy Policy and understand that I may opt out of MedicineNet's subscriptions at any time.

VIEW PATIENT COMMENTS
  • Bile Duct Cancer - Treatment

    What kind of treatment did you have for bile duct cancer?

    Post View 5 Comments
  • Bile Duct Cancer - Symptoms

    What symptoms did you have when you were diagnosed with bile duct cancer?

    Post View 3 Comments
  • Bile Duct Cancer - Stage

    What stage is your bile duct cancer?

    Post View 4 Comments

Health Solutions From Our Sponsors