Types of bile duct cancer

Bile duct cancer is also called cholangiocarcinoma. Doctors can group bile duct cancer in different ways. The most common way is by looking at where the cancer started.

Doctors can also group bile duct cancer based on the type of cell that the cancer started in.

Types of bile duct cancer by where the cancer starts

There are 3 types of bile duct cancer depending on where they start:

  • intrahepatic bile duct cancers
  • perihilar bile duct cancers
  • distal extrahepatic bile duct cancers

This video shows where the bile ducts are and the different areas where bile duct cancer can develop. The video is 1 minute and 30 seconds long.

Intrahepatic bile duct cancers

Intrahepatic bile duct cancers start inside the liver. There are a number of small bile ducts that join together inside the liver to form the left and right hepatic ducts. 

Diagram showing the position of the intrahepatic bile ducts

This type of bile duct cancer is sometimes confused with a different type of cancer that starts in liver cells. These are called hepatocellular carcinoma.

Perihilar bile duct cancers

These are cancers that develop just outside the liver, including where the left and right hepatic ducts join together. They are also called Klatskin tumours or hilar cancers. 

Diagram showing the position of the perihilar bile ducts

Distal extrahepatic bile duct cancers

These are cancers that start in the bile ducts below the perihilar region near the bowel. 

Diagram showing the position of the distal bile ducts

Types of bile duct cancer by the type of cell that the cancer started in

More than 9 in every 10 bile duct cancers (more than 90%) start in the cells that line the bile ducts. These are called epithelial cells. This type of cancer is called epithelial adenocarcinoma (add-en-oh-car-sin-oh-ma). 

There are a number of other types of bile duct cancer. These types of bile duct cancer are very rare and include:

  • squamous cell carcinoma
  • sarcoma
  • lymphoma

Non cancerous bile duct tumours

Non cancerous (benign) tumours can also grow in the bile duct. They include bile duct adenoma and hamartomas.

Last reviewed: 
11 Oct 2021
Next review due: 
11 Oct 2024
  • Guidelines for the diagnosis and treatment of cholangiocarcinoma: an update
    S Khan and others 
    Gut, 2012. Volume 61, Issue 12, Pages 1657-69

  • Biliary cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow up
    JW Valle and others
    Annals of Oncology, 2016. Volume 27, Pages 28-37

  • Cholangiocarcinoma 2020: the next horizon in mechanisms and managements
    JM Banales and others
    Nature reviews gastroenterology & hepatology, 2020. Vol 17, Pages 557-588

  • Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the study of cholangiocarcinoma (ENS-CCA)
    J Banales and others
    Nature Reviews gastroenterology & hepatology, 2016. Vol13. Pages 261-280

  • Benign inflammatory pseudotumour of the biliary tract masquerading as a Klatskin tumour
    PJ Worley and others
    HPB Oxford, 2001. Vol 3, Issue 2. Pages 179-181

  • The information on this page is based on literature searches and specialist checking. We used many references and there are too many to list here. Please contact patientinformation@cancer.org.uk with details of the particular issue you are interested in if you need additional references for this information.

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