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Extrahepatic Bile Duct Cancer Treatment (PDQ®)

Patient Version

Stages of Extrahepatic Bile Duct Cancer

After extrahepatic bile duct cancer has been diagnosed, tests are done to find out if cancer cells have spread within the bile duct or to other parts of the body.

The process used to find out if cancer has spread within the extrahepatic bile duct or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

Extrahepatic bile duct cancer may be staged following a laparotomy. A surgical incision is made in the wall of the abdomen to check the inside of the abdomen for signs of disease and to remove tissue and fluid for examination under a microscope. The results of the diagnostic imaging tests, laparotomy, and biopsy are viewed together to determine the stage of the cancer. Sometimes, a laparoscopy will be done before the laparotomy to see if the cancer has spread. If the cancer has spread and cannot be removed by surgery, the surgeon may decide not to do a laparotomy.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if extrahepatic bile duct cancer spreads to the liver, the cancer cells in the liver are actually extrahepatic bile duct cancer cells. The disease is metastatic extrahepatic bile duct cancer, not liver cancer.

There are two staging systems for extrahepatic bile duct cancer.

Extrahepatic bile duct cancer has two staging systems. The staging system used depends on where in the extrahepatic bile duct the cancer first formed.

  • Perihilar or proximal extrahepatic bile duct tumors (perihilar bile duct tumors) form in the area where the bile duct leaves the liver. This type of tumor is also called a Klatskin tumor.
    Klatskin tumor; drawing shows cancer in the common hepatic duct, the area where the right and left hepatic duct meet. Also shown are the liver and gallbladder.
    Klatskin tumor. A tumor that forms in the common hepatic duct, the area where the right and left hepatic duct meet.
  • Distal extrahepatic bile duct tumors (distal bile duct tumors) form in the area where the bile duct empties into the small intestine.

The following stages are used for perihilar extrahepatic bile duct cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, abnormal cells are found in the innermost layer of tissue lining the perihilar bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I

In stage I, cancer has formed in the innermost layer of the wall of the perihilar bile duct and has spread into the muscle and fibrous tissue of the wall.

Stage II

In stage II, cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or to the liver.

Stage III

Stage III is divided into stages IIIA and IIIB.

Stage IV

Stage IV is divided into stages IVA and IVB.

  • Stage IVA: The tumor may have spread to nearby lymph nodes and has spread to one or more of the following:
    • the main part of the portal vein or both branches of the portal vein;
    • the hepatic artery;
    • the right and left hepatic ducts;
    • the right hepatic duct and the left branch of the hepatic artery or of the portal vein;
    • the left hepatic duct and the right branch of the hepatic artery or of the portal vein.
  • Stage IVB: The tumor has spread to other parts of the body, such as the liver.

The following stages are used for distal extrahepatic bile duct cancer:

Stage 0 (Carcinoma in Situ)

In stage 0, abnormal cells are found in the innermost layer of tissue lining the distal bile duct. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I

In stage I, cancer has formed. Stage I is divided into stages IA and IB.

Stage II

Stage II is divided into stages IIA and IIB.

Stage III

In stage III, cancer has spread to the large vessels that carry blood to the organs in the abdomen. Cancer may have spread to nearby lymph nodes.

Stage IV

In stage IV, cancer has spread to other parts of the body, such as the liver or lungs.

Extrahepatic bile duct cancer can also be grouped according to how the cancer may be treated. There are two treatment groups:

Localized (and resectable)

The cancer is in an area where it can be removed completely by surgery.

Unresectable, recurrent, or metastatic

Unresectable cancer cannot be removed completely by surgery. Most patients with extrahepatic bile duct cancer have unresectable cancer.

Recurrent cancer is cancer that has recurred (come back) after it has been treated. Extrahepatic bile duct cancer may come back in the bile duct or in other parts of the body.

Metastasis is the spread of cancer from the primary site (place where it started) to other places in the body. Metastatic extrahepatic bile duct cancer may have spread to nearby blood vessels, the liver, the common bile duct, nearby lymph nodes, other parts of the abdominal cavity, or to distant parts of the body.

  • Updated: October 31, 2013