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Cholangiocarcinoma

Definition

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

Alternative Names

Bile duct cancer

Causes

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

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

Both men and women are affected. Most patients are older than 65.

Risks of this condition include:

  • Bile duct (choledochal) cysts
  • Chronic biliary and liver inflammation
  • History of infection with the parasitic worm, liver flukes
  • Primary sclerosing cholangitis
  • Ulcerative colitis

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

Symptoms

Symptoms may include any of the following:

Exams 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:

  • Abdominal CT scan
  • Abdominal ultrasound
  • CT scan-directed biopsy
  • Cytology of samples from the bile duct
  • Endoscopic retrograde cholangiopancreatography (ERCP)
  • Magnetic resonance cholangiopancreatography (MRCP)
  • Percutaneous transhepatic cholangiogram (PTCA)

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. Often the cancer has already spread locally or to another area of the body by the time it is diagnosed. As a result, surgery to cure the cancer is not possible.

Chemotherapy or radiation may be given after surgery to decrease the risk of the cancer returning. But 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.

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

Outlook (Prognosis)

Completely removing the tumor allows approximately 1 in 5 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, but rarely beyond 5 years.

Possible Complications


  • Infection
  • Liver failure
  • Spread (metastasis) of tumor to other organs

When to Contact a Medical Professional

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

References

National Cancer Institute: PDQ Extrahepatic Bile Duct Cancer Treatment. Bethesda, MD: National Cancer Institute. Date last modified 3/13/2014. Available at: http://cancer.gov/cancertopics/pdq/treatment/bileduct/HealthProfessional. Accessed May 29, 2014.

National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology (NCCN Guidelines): Hepatobiliary cancers. Version 2.2014. Available at: http://www.nccn.org/professionals/physician_gls/pdf/hepatobiliary.pdf. Accessed May 29, 2014.

Review Date:5/29/2014
Reviewed By:Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital, Boston, MA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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Outcome Data

No data available for this condition/procedure.

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