What is the definition of Cholangiocarcinoma?

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

What are the alternative names for Cholangiocarcinoma?

Bile duct cancer

What are the causes for Cholangiocarcinoma?

The exact cause of CCA isn't known. However, many of these tumors are already quite advanced by the time they are found.

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

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

People with the following health problems may have a higher chance of developing CCA:

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

What are the symptoms for Cholangiocarcinoma?

Symptoms of CCA may include any of the following:

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

What are the current treatments for Cholangiocarcinoma?

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. 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.

In select cases, a liver transplant can be tried.

Endoscopic therapy with stent placement can temporarily relieve blockages in the biliary ducts. This may also relieve jaundice when the tumor cannot be removed.

What is the outlook (prognosis) for Cholangiocarcinoma?

Completely removing the tumor allows some people to survive with the possibility of complete cure.

If the tumor cannot be completely removed, a cure is generally not possible. With treatment, about one half of affected people live a year, and about one half live longer, but rarely beyond 5 years.

Hospice is often a good resource for people with CCA that cannot be cured.

What are the possible complications for Cholangiocarcinoma?

Complications of CCA include:

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

When should I contact a medical professional for Cholangiocarcinoma?

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

Digestive system
Bile pathway


National Cancer Institute website. Bile duct cancer (cholangiocarcinoma) treatment (PDQ) -- health professional version. www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq. Updated September 23, 2020. Accessed November 9, 2020.

Rajkomar K, Koea JB. Intrahepatic cholangiocarcinoma. In: Jarnagin WR, ed. Blumgart's Surgery of the Liver, Biliary Tract and Pancreas. 6th ed. Philadelphia, PA: Elsevier; 2017:chap 50.

Rizvi SH, Gores GJ. Tumors of the bile ducts, gallbladder, and ampulla. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 69.

  • Condition: Liver Tumors
  • Journal: Chirurgia (Bucharest, Romania : 1990)
  • Treatment Used: Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy Procedure
  • Number of Patients: 18
  • Published —
The aim of this study was to evaluate the use of the Associating Liver Partition and Portal vein ligation for Staged hepatectomy (ALPPS) procedure for massive liver tumors.