Distal extrahepatic bile duct cancer stages

Bile duct cancer is also known as cholangiocarcinoma. There are different types of bile duct cancer depending on where it starts. Distal extrahepatic bile duct cancer starts in the bile ducts near the bowel. 

Diagram showing the position of the distal bile ducts

There are different ways of staging distal bile duct cancer. In the UK, doctors use the:

  • number staging system
  • TNM system

The stage of a cancer tells you how big the cancer is and whether it has spread. This is important because your doctor uses this information to decide which treatment you need. Your doctor looks at your test results to work out the stage.

The number staging system

The number staging system divides distal bile duct cancer into 4 main stages, from 1 to 4.

Stage 1

This means the cancer has grown less than 5mm into the bile duct wall. This is the same as T1, N0, M0 in the TNM staging system.

Stage 2

This is split into 2 groups:

Stage 2A means the cancer has either:

  • grown less than 5mm into the bile duct wall. And there are cancer cells in 1 to 3 of the nearby lymph nodes. This is the same as T1, N1, M0
  • grown 5 to 12mm into the bile duct wall. But there are no cancer cells in the nearby lymph nodes. This is the same as T2, N0, M0

Stage 2B means the cancer is one of the following:

  • between 5 and 12mm into the bile duct wall. And there are cancer cells in 1 to 3 of the nearby lymph nodes. This is the same as T2, N1, M0
  • more than 12mm into the bile duct wall. But there are no cancer cells in the nearby lymph nodes. This is the same as T3, N0, M0
  • more than 12mm into the bile duct wall. And there are cancer cells in 1 to 3 of the nearby lymph nodes. This is the same as T3, N1, M0

Stage 3

This is split into 2 groups:

Stage 3A means the cancer is one of the following:

  • less than 5mm into the bile duct wall. And there are cancer cells in 4 or more of the nearby lymph nodes. This is the same as T1, N2, M0
  • between 5 to 12mm into the bile duct wall. And there are cancer cells in 4 or more of the nearby lymph nodes. This is the same as T2, N2, M0
  • more than 12mm into the bile duct wall. And there are cancer cells in 4 or more of the nearby lymph nodes. This is the same as T3, N2, M0

Stage 3B means the cancer is one of the following:

  • in the main blood vessels in the tummy (abdomen). But there are no cancer cells in the nearby lymph nodes. This is the same as T4, N0, M0
  • in the main blood vessels in the abdomen. And there are cancer cells in 1 to 3 of the nearby lymph nodes. This is the same as T4, N1, M0
  • in a main blood vessel near the bile ducts. And there are cancer cells in 4 or more of the nearby lymph nodes. This is the same as T4, N2, M0

Stage 4

This means the cancer has spread away from the area around the bile ducts. The most common places for distal bile duct cancer to spread to include the liver and the lungs.

This is the same as any T, any N, M1 in the TNM staging system.

The TNM staging system

TNM stands for Tumour, Node and Metastasis. The system describes: 

  • the size of the primary tumour (T)
  • whether the cancer has spread to the lymph nodes (N)
  • whether the cancer has spread to a different part of the body (M)

Tumour (T) stages

There are 4 main T stages for distal bile duct cancer. They are numbered from T1 to T4:

T1 means the tumour is inside the bile duct and has grown less than 5mm into the bile duct wall.

T2 means the tumour has grown between 5 and 12 mm into the bile duct wall.

T3 means the tumour has grown more than 12mm into the bile duct wall.

T4 means the tumour is growing into a main blood vessel near the bile ducts.

Node (N) stages

There are 3 stages:

N0 means there are no cancer cells in the nearby lymph nodes.

N1 means there are cancer cells in 1 to 3 of the nearby lymph nodes.

N2 means there are cancer cells in 4 or more of the nearby lymph nodes.

Metastasis (M) stages

There are two M stages:

M0 means there is no signs that the cancer has spread.

M1 means the cancer has spread to other parts of the body. Common places for perihilar bile duct cancer to spread include the lungs and parts of the liver further away from the bile ducts.

Treatment options for distal bile duct cancer

The stage of the cancer helps your doctor decide which treatment is best for. Treatment also depends on:

  • where your cancer is
  • your general health and level of fitness
  • if there are gene Open a glossary item changes in the cancer cells

You may have surgery if you have an early stage distal bile duct cancer. Your doctor usually removes part of your pancreas, your gallbladder and the first part of the small bowel. This is called a pancreaticoduodenectomy.

This is a major operation. Your doctor will make sure that you are well enough to have it.

Unfortunately, most bile duct cancers are usually advanced by the time they are diagnosed. This means you might not be able to have the cancer removed. Your doctor might suggest other treatments to reduce your symptoms and help you feel better. This can include chemotherapy or putting a small tube (stent) in the bile duct. The stent opens up a bile duct that is blocked by the cancer. It means that bile can start to flow again.

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

  • Cancer: Principles and Practice of Oncology (11th edition)
    VT DeVita, TS Lawrence, SA Rosenberg
    Wolters Kluwer, 2019

  • Guidelines for the management and treatment of cholangiocarcinoma: an update
    SA Khan and others  
    Gut, 2012. Vol 61, issue 12.

  • Biliary cancer: ESMO clinical practice guidelines for diagnosis, treatment and follow up
    JW Valle and others
    Annals of oncology, 2016. Vol 27, Supplement 5. 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

Last reviewed: 
25 Jan 2022
Next review due: 
25 Jan 2022

Related links