Bile Duct Cancer Facts

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Last updated and reviewed on: August 17, 2026

Bile Duct Cancer Facts

Bile duct cancer is a rare cancer that develops in the tubes responsible for carrying bile through the liver and digestive system. Another name for bile duct cancer is cholangiocarcinoma.

What are the three types of bile duct cancer?

Doctors classify bile duct cancer according to where it begins.

Intrahepatic bile duct cancer

Intrahepatic cholangiocarcinoma develops in bile ducts located inside the liver. Because it develops within the liver, it may sometimes be confused with other types of liver tumors when it is first discovered.

Perihilar bile duct cancer

Perihilar cholangiocarcinoma develops where the right and left hepatic ducts leave the liver and join together. It is sometimes called a Klatskin tumor. Perihilar tumors are the most common type of bile duct cancer.

Distal bile duct cancer

Distal cholangiocarcinoma develops farther down the bile duct, closer to the small intestine and pancreas. Because of its location, surgery for distal bile duct cancer may involve removing parts of several nearby organs.

Is bile duct cancer the same as liver cancer?

No. Intrahepatic bile duct cancer develops inside the liver, but it starts in the cells lining the bile ducts rather than in the main liver cells. The most common primary liver cancer is hepatocellular carcinoma. Intrahepatic cholangiocarcinoma is a separate disease and is treated differently.

Is bile duct cancer the same as gallbladder cancer?

No. Both cancers occur within the biliary system, but gallbladder cancer begins in the gallbladder while cholangiocarcinoma begins in the bile ducts.

THE BASICS: Bile duct cancer, or cholangiocarcinoma, begins in the tubes that carry bile from the liver to the small intestine. The three main types are intrahepatic, perihilar, and distal bile duct cancer. The location of the tumor plays an important role in staging and treatment.

What are the bile ducts?

The bile ducts are a network of small tubes that carry bile from the liver and gallbladder to the small intestine. Bile is a yellow-green digestive fluid produced by the liver. It helps the body digest and absorb fats and fat-soluble vitamins.

How does the biliary system work?

Bile is continuously produced by the liver. Tiny bile ducts inside the liver collect bile and join together to form increasingly larger ducts. The right and left hepatic ducts eventually leave the liver and join to form the common hepatic duct. The gallbladder connects to the common hepatic duct through the cystic duct. Together, the common hepatic duct and cystic duct connect to the common bile duct. The common bile duct travels toward the small intestine, passing near or through the pancreas before emptying into the first part of the small intestine, called the duodenum.

What does bile do?

Bile helps your body:

  • Break down dietary fats

  • Absorb fat-soluble vitamins such as vitamins A, D, E, and K

  • Remove certain waste products from the body

  • Eliminate bilirubin, a substance produced when old red blood cells are broken down

How can a bile duct tumor affect the body?

A tumor can narrow or block a bile duct. When bile cannot drain normally, bilirubin can build up in the bloodstream. This can cause:

  • Yellowing of the skin and eyes

  • Dark urine

  • Pale or clay-colored stool

  • Itchy skin

A blocked bile duct can also cause infection, inflammation, liver problems, and digestive symptoms.

Where does bile duct cancer develop?

Bile duct cancer begins in cells lining the bile ducts. Its location determines which type of cholangiocarcinoma you have:

  • Intrahepatic. Inside the liver

  • Perihilar. Just outside the liver where major ducts join

  • Distal. Farther down the common bile duct near the pancreas and small intestine

THE BASICS: The bile ducts carry bile from the liver and gallbladder to the small intestine. Bile helps digest fats. If bile duct cancer blocks this system, bile can build up and cause symptoms such as jaundice, itching, dark urine, and pale stool.

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