Your bile ducts are tubes that carry bile from your liver and gallbladder to your small intestines. Bile is a fluid produced by your liver and stored in your gallbladder. It helps your body break down fatty acids.

Bile duct cancer, or cholangiocarcinoma, is a rare type of cancer that forms in these tubes. It makes up about 3 percent of gastrointestinal cancers. Because your bile ducts are deep inside your body and there are no reliable screening tests, bile duct cancer usually isn’t detected until it’s grown enough to cause symptoms.

Doctors divide bile duct cancer into stages depending on how much it has grown and whether it has spread to other tissues or lymph nodes. Keep reading to learn more about how doctors stage bile duct cancer.

The primary staging system for bile duct cancer is the American Joint Committee on Cancer (AJCC)’s TNM system.

The TNM system stages cancer based on:

  • Tumor size. How large has the tumor grown?
  • Nodes. Has the cancer spread to nearby lymph nodes?
  • Metastasis. Has the cancer spread to distant tissues?

This staging system divides bile duct cancer into stages 0 to 4, with a higher number corresponding with more advanced cancer based on the results of:

  • a biopsy
  • imaging
  • a physical exam

Staging also considers where the tumor develops. The three categories are:

  • Intrahepatic bile duct cancer. Cancer of the part of your bile duct inside your liver.
  • Perihilar (hilar) bile duct cancer. Cancer in an area just outside your liver where your left and right hepatic ducts meet (the hilum).
  • Distal (extrahepatic) bile duct cancer. Cancer below the hilum.

Knowing where your cancer is and what stage it’s in helps doctors develop the best treatment plan and predict your chances of survival.

Illustration depicting the anatomy of bile duct cancerShare on Pinterest
Anatomy of the bile duct, liver, pancreas, and duodenum, showing locations of bile duct, intrahepatic and extrahepatic cancers. Illustration by Sophia Smith

The TNM system stages intrahepatic bile duct cancer as follows:

StageDescription
0Tis. The tumor is contained in the innermost layer of your bile duct called the mucosa.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IAT1a. The tumor is less than 2 inches across and has not reached your blood vessels.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IBT1b. The tumor is more than 2 inches across and has not reached your blood vessels.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIT2. The tumor has grown into nearby blood vessels OR there are at least 2 tumors.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIAT3. The tumor has grown through the outer lining of your abdominal organs called the visceral peritoneum.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIBT4. The cancer has spread to structures outside your liver.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.

OR

Any T. The cancer has grown to any size.
N1. The cancer has spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IVAny T. The cancer has grown to any size.
Any N. The cancer has or hasn’t spread to lymph nodes.
M1. The cancer has spread to distant tissues such as your lungs or bones.

The TNM system stages perihilar bile duct cancer as follows:

StageDescription
0Tis. The tumor is contained in the innermost layer of your bile duct called the mucosa.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IT1. The cancer has spread to the deeper layers of your bile duct, such as the muscle or connective tissue.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIT2a. The cancer has grown through your bile duct and into fatty tissue OR
T2b. The cancer has grown into liver tissues.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIAT3. The tumor has grown into branches of your portal vein or the hepatic artery, the main blood vessels of your liver.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIBT4. The cancer is growing into your portal vein or common hepatic artery OR into branches of these blood vessels on the left and right OR it’s growing into other bile ducts on either the left or right and a major blood vessel on the other side.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIICAny T. The cancer is any size.
N1. The cancer has spread to 1 to 3 lymph nodes.
M0. The cancer has not spread to distant tissues.
IVAAny T. The cancer is any size.
N2. The cancer has spread into 4 or more lymph nodes.
M0. The cancer has not spread to distant tissues.
IVBAny T. The cancer is any size.
Any N. The cancer has spread to any number of lymph nodes or no lymph nodes.
M1. The cancer has spread to distant organs, such as other parts of your liver, lungs, or bones.

The AJCC TNM system stages distal bile duct cancer as follows:

StageDescription
0Tis. The tumor is contained in the innermost layer of your bile duct called the mucosa.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IT1. The cancer has grown less than 0.2 inches into the bile duct wall.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIAT2. The cancer has grown from 0.2 inches to 0.5 inches into the bile duct wall.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.

OR

T1. The cancer has grown less than 0.2 inches into the bile duct wall.
N1. The cancer has spread to 1 to 3 lymph nodes.
M0. The cancer has not spread to distant tissues.
IIBT3. The cancer has grown more than 0.5 inches into the bile duct wall.
N0. The cancer has not spread to nearby lymph nodes.
M0. The cancer has not spread to distant tissues.

OR

T2 or T3. The cancer has grown at least 0.2 inches into the bile duct wall.
N1. The cancer has spread to 1 to 3 nearby lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIAT1, T2, or T3. The cancer has grown into the bile duct wall to any degree.
N2. The cancer has spread to 4 or more lymph nodes.
M0. The cancer has not spread to distant tissues.
IIIBT4. The cancer has grown into nearby blood vessels.
Any N. The cancer may or may not have grown into lymph nodes.
M0. The cancer has not spread to distant tissues.
IVAny T. The cancer has grown to any degree into the bile duct wall.
Any N. The cancer may or may not have grown into lymph nodes.
M1. The cancer has spread to distant organs such as the inner lining of your abdomen, lungs, or liver.

A resectable cancer is a tumor that can be removed surgically. Unresectable cancers are those that can’t be removed surgically because they’ve spread too far or are in a difficult-to-access location. Most bile duct cancers are unresectable by the time they’re detected and diagnosed.

Unfortunately, few bile duct cancers are caught in the early stages. Your bile ducts are deep inside your body. The tumors can’t be felt, and there are no reliable screening tests. By the time symptoms develop, the tumor has usually progressed beyond the point where it can be surgically removed.

When symptoms do present, they may include:

These symptoms can have many different causes unrelated to bile cancer. However, it’s important to visit a doctor if you have any of these warning signs with no obvious cause.

The 5-year survival rate is 15 to 25 percent when diagnosed in the early stages but drops to 2 percent if the cancer spreads to distant body parts.

Doctors most often use the AJCC TNM system to stage bile duct cancer. This system stages bile cancers differently depending on where the cancer develops in your bile ducts.

Knowing how far along your cancer has progressed can help you and your medical team work together to build the best treatment plan.