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Expert-reviewed information summary about the treatment of extrahepatic bile duct cancer.

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A.D.A.M. Medical Encyclopedia. Atlanta (GA): A.D.A.M.; 2011.

A.D.A.M. Medical Encyclopedia.

Cholangiocarcinoma

Bile duct cancer

Last reviewed: June 2, 2010.

Cholangiocarcinoma is a cancerous (malignant) growth in one of the ducts that carries bile from the liver to the small intestine.

Causes, incidence, and risk factors

Cancerous tumors of the bile ducts are usually slow-growing and do not spread (metastasize) quickly. However, many of these tumors are already advanced by the time they are found.

A cholangiocarcinoma may start anywhere along the bile ducts. These tumors block off the bile ducts.

They affect both men and women. Most patients are older than 65.

Risks for this condition include:

Cholangiocarcinoma is rare. It occurs in approximately 2 out of 100,000 people.

Symptoms

Signs and tests

Your health care provider will perform a physical exam. Tests will be done to check for a tumor or blockage in the bile duct. These may include:

Blood tests that may be done include:

Treatment

The goal is to treat the cancer and the blockage it causes. When possible, surgery to remove the tumor is the treatment of choice and may result in a cure. If the tumor is large, the entire liver may need to be removed and a liver transplant will be needed. However, often the cancer has already spread by the time it is diagnosed.

Chemotherapy or radiation may be given after surgery to decrease the risk of the cancer returning. However, the benefit of this treatment is not certain.

Endoscopic therapy with stent placement can temporarily relieve blockages in the biliary ducts and relieve jaundice in patients when the tumor cannot be removed. Laser therapy combined with light-activated chemotherapy medications is another treatment option for those with blockages of the bile duct.

Support Groups

You can ease the stress of illness by joining a support group with members who share common experiences and problems (see cancer - support group).

Hospice is often a good resource for patients with cholangiocarcinoma that cannot be cured.

Expectations (prognosis)

Completely removing the tumor allows 30 - 40% of patients to survive for at least 5 years, with the possibility of a complete cure.

If the tumor cannot be completely removed, a cure is generally not possible. With treatment, about half of these patients live a year, and about half live longer.

Complications

  • Infection

  • Liver failure

  • Spread (metastasis) of tumor to other organs

Calling your health care provider

Call your health care provider if you have jaundice or other symptoms of cholangiocarcinoma.

References

  1. Lewis RL. Liver and biliary tract tumors. In Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 206.

Review Date: 6/2/2010.

Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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What works?

  • Insufficient evidence to support or refute glucocorticosteroids for primary sclerosing cholangitis Insufficient evidence to support or refute glucocorticosteroids for primary sclerosing cholangitis
    Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and, ultimately, liver cirrhosis. Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of patients. Several therapeutic modalities have been proposed for primary sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis with liver cirrhosis.
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