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

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:

• 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

• Chills
• Clay-colored stools
• Fever
• Itching
• Loss of appetite
• Pain in the upper right abdomen that may radiate to the back
• Weight loss
• Yellowing of the skin (jaundice)

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:

• 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:

• Liver function tests (especially alkaline phosphatase or bilirubin levels)

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 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.

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,Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 202.
2. National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Hepatobiliary cancers. 2012. Version 2.2012.

Review Date: 6/5/2012.

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. Health Solutions, Ebix, Inc.