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Definition

Cholangiocarcinoma is the growth of cancer cells in the bile duct. The bile duct are a number of branching tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a fluid that helps digest food and eliminate waste from the body.

Cholangiocarcinomas can be divided by their location:

  • Perihilar—develops at the point where the branches of the bile duct join and leave the liver (most common form)
  • Intrahepatic—develops in the bile duct branches that are located in the liver
  • Distal—develops in the area of the bile duct that is closer to the small intestine
Common Bile Duct
common bile duct
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Causes

Cancer occurs when cells in the body divide without control or order. Normally, cells divide in a regulated manner. If cells keep dividing uncontrollably when new cells are not needed, a mass of tissue forms, called a growth or tumor. The term cancer refers to malignant growths. These growths can invade nearby tissues. Cancer that has invaded nearby tissues can then spread to other parts of the body.

Defects on specific genes may lead to the growth of cancer cells in the bile duct. Other causes may be related to a combination of genetics and environment.

Risk Factors

Cholangiocarcinoma is more common in people 65 years and older. Other factors that may increase your chance of cholangiocarcinoma include:

  • Having a chronic condition that causes inflammation of the bile duct such as primary sclerosing cholangitis, ulcerative colitis , bile duct stones, choledochal cysts
  • Overweight or obesity
  • Family history of bile duct cancer
  • Cirrhosis of the liver or another condition that affects the liver or bile duct such as polycystic liver disease, Caroli syndrome
  • Exposure to a radioactive substance or toxic chemicals
  • Infection by parasites called liver flukes (more common in Asian countries)

Other possible risk factors are:

Symptoms

In the early stages of the cancer, there may not be any symptoms. As the cancer grows, symptoms may include:

  • Yellowing of the skin and whites of the eyes—jaundice
  • Abdominal pain
  • Fever
  • Weakness, fatigue
  • Decrease in appetite, weight loss
  • Itchy skin
  • Dark urine, pale stool

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. Blood tests will help to evaluate how the liver and gallbladder are working. These tests may also help to find tumor marker, indicators of cancer in the body.

Imaging tests can evaluate the bile duct and surrounding structures. These may include:

Other tests may may include:

  • Laparoscopy—thin tube with a lighted camera on one end to examine the bile duct
  • Biopsy—a sample of bile duct cells is removed and examined

The physical exam combined with all of your test results, will help to determine the stage of cancer you have. Staging is used to guide your treatment plan. Like other cancers, cholangiocarcinoma is staged from I-IV. Stage I is a very localized cancer, while stage IV indicates a spread to other parts of the body.

Treatment

The treatment plan depends on the stage of the cancer and your overall health.

Surgery

Surgery may be done to try to remove the cancer. The bile duct reaches into many organs. Surgery may involve more than one organ. For example:

  • Perihilar bile duct cancer—may include the removal of part of the liver, bile duct, and gall bladder
  • Intrahepatic bile duct cancer—may include the removal of part of the liver or an entire lobe
  • Distal bile duct cancer—may include the removal of part of the pancreas and small intestine

If the cancer cannot be removed, other types of surgery may be done to relieve symptoms. A small tube may be placed inside of a cancerous bile duct to allow bile to flow through it.

Radiation Therapy

Radiation therapy is used to kill cancer cells and shrink tumors. Different forms include:

  • External radiation therapy—radiation directed at the tumor from a source outside the body
  • Internal radiation therapy—radioactive materials placed into the body in or near the cancer cells

Radiation may be done along with surgery. It may also be the main treatment if cancer cannot be removed.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. This treatment may be used before or after surgery. If the tumor cannot be removed, chemotherapy may be given, either alone or in combination with radiation therapy.

Clinical Trials

Clinical trials may be recommended if treatment options are limited for your type of cancer. These trials could offer treatments that are not currently available to most. Talk to your doctor about these options.

Prevention

To help reduce your chance of cholangiocarcinoma:

Revision Information

  • American Cancer Society

    http://www.cancer.org

  • Cholangiocarcinoma Foundation

    http://www.cholangiocarcinoma.org

  • BC Cancer Agency

    http://www.bccancer.bc.ca

  • Canadian Cancer Society

    http://www.cancer.ca

  • Bile duct cancer (cholangiocarcinoma. American Cancer Society website. Available at: http://www.cancer.org/acs/groups/cid/documents/webcontent/003084-pdf.pdf. Updated March 2, 2011. Accessed June 18, 2013.

  • Bile duct cancer (cholangiocarcinoma). Johns Hopkins Medicine website. Available at: http://www.hopkinsmedicine.org/liver%5Ftumor%5Fcenter/conditions/bile%5Fduct%5Fcancer.html. Accessed June 18, 2013.

  • Bile duct cancer. Cancer.net website. Available at: http://www.cancer.net/cancer-types/bile-duct-cancer. Updated February 2012. Accessed June 18, 2013.

  • Cholangiocarcinoma. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated April 22, 2013. Accessed June 18, 2013.