About Cholangiocarcinoma

What is Cholangiocarcinoma?

Cholangiocarcinoma, also known as bile duct cancer, is cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid (bile). Bile is moved via the bile ducts from your liver and gallbladder to your small intestine to help digest fat in the food you eat.

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

  • Intrahepatic cholangiocarcinoma (ICC) 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

Tumors that develop outside of your liver tend to be small. Those in the liver can be small or large. In most cases, cholangiocarcinoma arises in those parts of the bile ducts that lie outside the liver. Rarely, the cancer can develop in ducts that are located within the liver.

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.

1For more information, visit the American Cancer Society web site on cholangiocarcinoma.