Types of bile duct cancer

Bile duct cancer is also called cholangiocarcinoma. There are 3 main types, depending on where in the bile ducts the cancer starts. They are:

  • intrahepatic bile duct cancer
  • perihilar bile duct cancer
  • distal bile duct cancer

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 cancer

There are a number of small bile ducts in the liver. These join together to make larger ducts called the right and left hepatic duct.

Cancer that starts in the bile ducts in the liver is called intrahepatic bile duct cancer.

Diagram showing the position of the intrahepatic bile ducts

Sometimes your doctor might find it difficult to tell if you have intrahepatic bile duct cancer, or cancer that started in the liver cells. Cancer that starts in the liver cells is called hepatocarcinoma. Your doctor might recommend taking a piece of tissue (biopsy) from the cancer to check which one it is.

Perihilar bile duct cancer

The perihilar bile ducts are outside the liver. They leave the liver just before the left and right hepatic duct meet. And stop where the cystic duct joins the common hepatic duct.

Cancer that starts in the perihilar bile ducts is called perihilar bile duct cancer. It can also be called a Klatskin tumour or hilar cancer.

Diagram showing the position of the perihilar bile ducts

Distal bile duct cancer

The distal bile ducts start where the cystic duct joins the common hepatic duct. This forms the common bile duct which takes bile to the small bowel.

Cancer that starts in this area is called distal bile duct cancer. 

Diagram showing the position of the distal bile ducts

Gene changes (mutations) in the cancer

Your doctor might check the bile duct cancer cells to see if they have certain gene Open a glossary item changes (mutations). These changes can help the cancer grow and divide. Knowing if the cancer has these changes can help your doctor decide on the best treatment for you. 

This is a type of personalised medicine.

Other types of bile duct cancer

The bile ducts are lined by a layer of tissue called epithelial tissue. There are different types of epithelial tissue cell. Most cancers of the bile duct start in epithelial cells that keep the tissue moist. These cancers are called adenocarcinomas.

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

  • squamous cell carcinoma Open a glossary item
  • sarcoma Open a glossary item
  • lymphoma Open a glossary item

Non cancerous bile duct tumours

Non cancerous (benign) tumours can also grow in the bile ducts. They include bile duct adenomas Open a glossary item and hamartomas. A bile duct hamartoma is where normal bile duct tissue overgrows to form a lump.

  • Cholangiocarcinoma
    PJ Brindley and others
    Nature Reviews Disease Primers, 2021, Volume 7, Article number 65

  • AJCC Cancer Staging Manual (8th edition)
    American Joint Committee on Cancer,
    Springer, 2017

  • Ross and Wilson Anatomy and Physiology in Health and Illness (14th edition)
    A Waugh and A Grant
    Elsevier Ltd, 2023

  • Benign biliary neoplasms and tumor precursors
    S Sarcognato and others
    Pathologica, 2021. Volume 113, Issue 3, Pages 147-157

  • EASL - ILCA Clinical Practice Guidelines on the management of intrahepatic cholangiocarcinoma
    European Association for the Study of the Liver
    Journal of Hepatology, 2023. Volume 79, Pages 181-208

  • 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.

Last reviewed: 
16 Jul 2024
Next review due: 
16 Jul 2027

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