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Bile duct cancer

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Malignant tumours of the extrahepatic bile duct

Malignant tumours are cancerous growths that have the potential to spread (metastasize) to other parts of the body. Extrahepatic bile duct cancers start in bile ducts outside the liver. They are either:

  • perihilar (hilar) bile duct cancers
    • These include cancers in the right and left hepatic ducts as they leave the liver and join to form the common hepatic duct, as well as above the origin of the cystic duct. They do not include cancers of the cystic duct.
    • They are also called proximal bile duct tumours or Klatskin tumours.
    • The perihilar area is the most common location of bile duct cancers.
  • distal bile duct cancers
    • These include cancers in the common bile duct between the junction of the cystic duct and the ampulla of Vater, near where the bile duct empties into the small intestine. They do not include cancers in these structures.
    • The distal area is the second most common location of bile duct cancers.

Intrahepatic bile duct cancer occurs in the bile ducts within the liver. This type of bile duct cancer is staged and treated somewhat differently than extrahepatic bile duct cancer.

The following information applies to extrahepatic bile duct tumours.


Adenocarcinoma is a type of cancer that starts in the gland cells that line the inside of the bile ducts. Adenocarcinomas are the most common type of bile duct cancers and account for over 90% of all malignant bile duct tumours. Adenocarcinomas are grouped into different subtypes based on how they look under the microscope or the types of characteristics they have.

  • papillary adenocarcinomas
  • intestinal-type adenocarcinomas
  • mucinous adenocarcinomas

Rare extrahepatic bile duct tumours

The following types of extrahepatic bile duct tumours are rare:

  • signet-ring cell carcinoma
  • clear cell carcinoma
  • adenosquamous carcinoma
  • squamous cell carcinoma
  • small (oat) cell carcinoma
  • undifferentiated carcinoma
  • neuroendocrine tumour
  • sarcoma


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