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What Is Bile Duct Cancer (Cholangiocarcinoma)?

Bile duct cancer is a rare disease in which malignant (cancer) cells form in the bile ducts. Bile duct cancer is also called cholangiocarcinoma.

A network of tubes, called ducts, connects the liver, gallbladder, and small intestine. This network begins in the liver where many small ducts collect bile (a fluid made by the liver to break down fats during digestion). The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder.

When food is being digested, bile stored in the gallbladder is released and passes through the cystic duct to the common bile duct and into the small intestine.

Types of bile duct cancer

There are two types of bile duct cancer:

  • Intrahepatic bile duct cancer: This type of cancer forms in the bile ducts inside the liver. Only a small number of bile duct cancers are intrahepatic. Intrahepatic bile duct cancers are also called intrahepatic cholangiocarcinomas.
    EnlargeAnatomy of the intrahepatic bile ducts; drawing shows the liver and the intrahepatic bile ducts, which include the right and left hepatic ducts. Also shown is the common hepatic duct, gallbladder, cystic duct, common bile duct, pancreas, and small intestine. An inset shows a cross section of a liver lobule with a network of bile ductules leading into a bile duct.
    Anatomy of the intrahepatic bile ducts. Intrahepatic bile ducts are a network of small tubes that carry bile inside the liver. The smallest ducts, called ductules, come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct from the gallbladder joins the common hepatic duct to form the common bile duct. The common bile duct passes through the pancreas and ends in the small intestine. Bile is made by the liver and stored in the gallbladder. When food is being digested, bile is released from the gallbladder and passes through the pancreas into the small intestine, where it helps digest fats.
  • Extrahepatic bile duct cancer: This type of cancer forms in the bile ducts outside the liver. The two types of extrahepatic bile duct cancer are perihilar bile duct cancer and distal bile duct cancer:
    • Perihilar bile duct cancer: This type of cancer is found in the area where the right and left bile ducts exit the liver and join to form the common hepatic duct. Perihilar bile duct cancer is also called a Klatskin tumor or perihilar cholangiocarcinoma.
    • Distal bile duct cancer: This type of cancer is found in the area where the ducts from the liver and gallbladder join to form the common bile duct. The common bile duct passes through the pancreas and ends in the small intestine. Distal bile duct cancer is also called extrahepatic cholangiocarcinoma.
    EnlargeAnatomy of the extrahepatic bile ducts; drawing shows the extrahepatic bile ducts, including the common hepatic duct (perihilar region) and the common bile duct (distal region). Also shown are the liver, right and left hepatic ducts, gallbladder, cystic duct, pancreas, and small intestine.
    Anatomy of the extrahepatic bile ducts. Extrahepatic bile ducts are small tubes that carry bile from the liver and gallbladder to the small intestine. They are made up of the common hepatic duct (perihilar region) and the common bile duct (distal region). Bile is made in the liver and flows through the common hepatic duct and the cystic duct to the gallbladder, where it is stored. When food is being digested, bile is released from the gallbladder and flows through the common bile duct and pancreas into small intestine.

Signs and symptoms of bile duct cancer

These and other signs and symptoms may be caused by bile duct cancer or by other conditions. Check with your doctor if you have any of the following:

  • jaundice (yellowing of the skin or whites of the eyes)
  • dark urine
  • clay colored stool
  • pain in the abdomen
  • fever
  • itchy skin
  • nausea and vomiting
  • weight loss for an unknown reason

There are no routine screening tests to check for bile duct cancer before signs and symptoms occur. To learn about tests that are used to diagnose bile duct cancer, see Bile Duct Cancer Diagnosis.

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