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, 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.

Ricks for this condition include:

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

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

Tests that show a tumor or blockage in the bile duct:

• Abdominal CT scan
• Abdominal ultrasound
• CT scan-directed biopsy
• Cytology
• Endoscopic retrograde cholangiopancreatography (ERCP)
• Percutaneous transhepatic cholangiogram (PTCA)

Blood tests that show abnormal function:

• Liver function tests (especially bilirubin)

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. 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 or surgery can clear blockages in the biliary ducts and relieve jaundice in patients when the tumor cannot be removed.

For patients with cancer that cannot be removed, radiation therapy may be beneficial. Chemotherapy may be added to radiation therapy or used when the tumor has spread. However, this is rarely effective.

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

Lillemoe KD. Tumors of the gallbladder, bile ducts, and ampulla. In: Feldman M, Friedman LS, Brandt LJ. Feldman: Sleisenger & Fordtran's Gastrointestinal and Liver Disease. 8th ed. Philadelphia, Pa: Saunders Elsevier: chap 66.