Overview

Cholangiocarcinoma is cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile. Bile ducts connect your liver to your gallbladder and to your small intestine. This condition, also known as bile duct cancer, is an uncommon form of cancer that occurs mostly in people older than age 50, though it can occur at any age.

Doctors divide cholangiocarcinoma into different types based on where the cancer occurs in the bile ducts:

  • Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver and is sometimes classified as a type of liver cancer.
  • Hilar cholangiocarcinoma occurs in the bile ducts just outside of the liver. This type is also called perihilar cholangiocarcinoma.
  • Distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine.

Cholangiocarcinoma is a type of tumor that is very difficult to treat.

Cholangiocarcinoma care at Mayo Clinic

Types

Symptoms

Signs and symptoms of cholangiocarcinoma include:

  • Yellowing of your skin and the whites of your eyes (jaundice)
  • Intensely itchy skin
  • White-colored stools
  • Fatigue
  • Abdominal pain
  • Unintended weight loss

When to see a doctor

See your doctor if you have persistent fatigue, abdominal pain, jaundice, or other signs and symptoms that bother you. He or she may refer you to a specialist in digestive diseases (gastroenterologist).

Causes

Cholangiocarcinoma occurs when cells in the bile ducts develop changes (mutations) in their DNA — the material that provides instructions for every chemical process in your body. DNA mutations cause changes in the instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of cancerous cells. It's not clear what causes the genetic mutations that lead to cancer.

Risk factors

Factors that may increase your risk of cholangiocarcinoma include:

  • Primary sclerosing cholangitis. This disease causes hardening and scarring of the bile ducts.
  • Chronic liver disease. Scarring of the liver caused by a history of chronic liver disease increases the risk of cholangiocarcinoma.
  • Bile duct problems present at birth. People born with a choledochal cyst, which causes dilated and irregular bile ducts, have an increased risk of cholangiocarcinoma.
  • A liver parasite. In areas of Southeast Asia, cholangiocarcinoma is associated with liver fluke infection, which can occur from eating raw or undercooked fish.
  • Older age. Cholangiocarcinoma occurs most often in adults over age 50.
  • Smoking. Smoking is associated with an increased risk of cholangiocarcinoma.

Prevention

There's no way to prevent cholangiocarcinoma (bile duct cancer). But you may reduce your risk of the disease if you:

  • Stop smoking. Smoking is linked to an increased risk of cholangiocarcinoma. If you smoke, stop. If you've tried quitting in the past and haven't been successful, talk with your doctor about strategies to help you quit.
  • Reduce your risk of liver disease. Chronic liver disease is associated with an increased risk of cholangiocarcinoma. Some causes of liver disease can't be prevented, but others can. Do what you can to take care of your liver.

    For instance, to reduce your risk of liver inflammation (cirrhosis), drink alcohol in moderation, if at all. Maintain a healthy weight. When working with chemicals, follow the safety instructions on the container.

A study published in 2016 showed that aspirin use may help reduce the risk of developing cholangiocarcinoma. The study involved data on nearly 4,800 people. Further study is needed to be certain that long-term aspirin use is safe for cancer prevention.

Cholangiocarcinoma (bile duct cancer) care at Mayo Clinic

April 25, 2017
References
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