Cholangiocarcinoma is the growth of cancer cells in the bile duct. The bile ducts are a number of branching tubes that carry bile from the liver to the gallbladder and small intestine. Bile is a fluid that helps with food digestion and the elimination of waste from the body.

Cholangiocarcinomas are named by their location.

  • Perihilar—develops at the point where the branches of the bile duct join and leave the liver (most common form)
  • Intrahepatic—develops in the bile duct branches that are located in the liver
  • Distal—develops in the area of the bile duct that is closer to the small intestine

Common Bile Duct
common bile duct

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Cancer occurs when cells in the body divide without control or order. Normally, cells divide in a regulated manner. If cells keep dividing uncontrollably when new cells are not needed, a mass of tissue forms, called a growth or tumor. The term cancer refers to malignant growths. These growths can invade nearby tissues. Cancer that has invaded nearby tissues can then spread to other parts of the body.

Defects on specific genes may lead to the growth of cancer cells in the bile duct. Other causes may be related to a combination of genetics and environment.

Risk Factors

Cholangiocarcinoma is more common in people 65 years and older. Other factors that may increase your chances of cholangiocarcinoma:

  • Having a chronic condition that causes inflammation of the bile duct such as primary sclerosing cholangitis, ulcerative colitis, bile duct stones, choledochal cysts
  • Excess weight or obesity
  • Family history of bile duct cancer
  • Conditions that affect the liver or bile ducts such as cirrhosis of the liver, polycystic liver disease, or Caroli syndrome
  • Exposure to radioactive substances or toxic chemicals
  • Infection by parasites called liver flukes (more common in Asian countries)

Other possible risk factors are:


In the early stages of the cancer, there may not be any symptoms. As the cancer grows, symptoms may include:

  • Yellowing of the skin and whites of the eyes— jaundice
  • Abdominal pain
  • Fever
  • Weakness, fatigue
  • Decrease in appetite, weight loss
  • Itchy skin
  • Dark urine, pale stool


Your doctor will ask about your symptoms and medical history. A physical exam will be done. Blood tests will help to evaluate how the liver and gallbladder are working. These tests may also help to find tumor marker, indicators of cancer in the body.

Imaging tests can evaluate the bile duct and surrounding structures. These may include:

Other tests may may include:

  • Laparoscopy —thin tube with a lighted camera on one end to examine the bile duct
  • Biopsy —a sample of bile duct cells is removed and examined under a microscope

The physical exam combined with all of your test results, will help to determine the stage of cancer you have. Staging is used to guide your treatment plan. Like other cancers, cholangiocarcinoma is staged from I-IV (1-4). Stage I is a very localized cancer, while stage IV indicates a spread to other parts of the body.


The treatment plan depends on the stage of the cancer and your overall health.


Surgery may be done to try to remove the cancer. The bile duct reaches into many organs. Surgery may involve more than one organ. For example:

  • Perihilar bile duct cancer—may include the removal of part of the liver, bile duct, and gall bladder
  • Intrahepatic bile duct cancer—may include the removal of part of the liver or an entire lobe
  • Distal bile duct cancer—may include the removal of part of the pancreas and small intestine

If the cancer cannot be removed, other types of surgery may be done to relieve symptoms. A small tube or stent may be placed inside of a blocked bile duct to allow bile to flow through it.

Radiation Therapy

Radiation therapy is used to kill cancer cells and shrink tumors. Different forms include:

  • External radiation therapy—radiation directed at the tumor from a source outside the body
  • Internal radiation therapy—radioactive materials placed into the body in or near the cancer cells

Radiation may be done along with surgery. It may also be the main treatment if cancer cannot be removed.


Chemotherapy is the use of drugs to kill cancer cells. This treatment may be used before or after surgery. If the tumor cannot be removed, chemotherapy may be given, either alone or in combination with radiation therapy.

Clinical Trials

Clinical trials may be recommended if treatment options are limited for your type of cancer. These trials could offer treatments that are not currently available to most. Talk to your doctor about these options.


To help reduce your chances of cholangiocarcinoma:


American Cancer Society

The Cholangiocarcinoma Foundation


Canadian Cancer Society

Provincial Health Services Authority


Bile duct cancer. American Cancer Society website. Available at: Accessed January 8, 2018.

Bile duct cancer (cholangiocarcinoma). Cancer.Net—American Society of Clinical Oncology website. Available at: Updated February 2012. Accessed January 8, 2018.

Bile duct cancer (cholangiocarcinoma). Johns Hopkins Medicine website. Available at: Accessed January 8, 2018.

Cholangiocarcinoma and gallbladder cancer. EBSCO DynaMed Plus website. Available at: Updated August 2, 2017. Accessed January 8, 2018.

Last reviewed November 2018 by EBSCO Medical Review Board Mohei Abouzied, MD, FACP  Last Updated: 12/20/2014