Bile duct cancer, also known as cholangiocarcinoma, is a rare form of cancer that affects the bile ducts. Two bile ducts in the human body connect the liver and gallbladder to the small intestine. They are important for digestion.

Bile duct cancer can start either inside the liver — intrahepatic — or outside of the liver — extrahepatic. Though very similar, the two types of bile duct cancer have somewhat different survival outlooks and may present with slightly different symptoms.

Experts break bile duct cancer down into several different stages. This helps doctors explain a person’s outlook, as well as help guide their treatment options.

This article reviews the main symptoms of bile duct cancer, early warning signs, its symptoms by stage, and more.

According to the American Cancer Society (ACS), early stages of bile duct cancer do not tend to present with symptoms. When it does, it is usually due to a tumor blocking a bile duct.

The most common symptom of bile duct cancer is jaundice, which is a yellowing of the eyes and skin.

However, bile duct cancer can cause other symptoms, such as:

Bile duct cancer is difficult to catch early. This is partly because the bile ducts are hard to reach, buried deep within the abdomen. Doctors cannot feel for a lump or mass during a routine exam, nor can they see one on imaging scans.

There are also no reliable blood tests to check for bile duct cancer.

Finally, bile duct cancer typically does not show early warning signs or symptoms unless a tumor has blocked a bile duct.

Bile duct cancer is a rare form of cancer, which means that many of the associated symptoms are more likely the result of another condition affecting the liver.

Doctors typically describe cancer in terms of its stage.

Staging provides doctors with a way to easily describe the cancer’s progression, as well as by helping them determine the best available treatment options.

Bile duct cancer can be intrahepatic or extrahepatic. Extrahepatic includes perihilar and distal bile duct cancers.

The table below describes the stages for each type of bile duct cancer, and what symptoms may present at each stage, according to the National Cancer Institute (NCI) and ACS.

StagesIntrahepatic bile duct cancerPerihilar bile duct cancerDistal bile duct cancer Symptoms
1 Cancer has formed inside the tissue lining the intrahepatic bile duct. Substages are:
1A: The tumor is 5 centimeters (cm) or smaller.
1B: The tumor is larger than 5 cm.
Cancer has formed in the innermost layer of tissue lining the perihilar bile duct.
It has spread into the muscle layer or fibrous tissue layer of the perihilar bile duct wall.
Cancer has formed in the innermost layer of the lining of the bile duct.
It has spread fewer than 5 millimeters (mm) into the wall of the distal bile duct.
Symptoms are not likely to occur in these early stages.
2Either:
The tumor has spread through the wall of an intrahepatic bile duct and into a nearby blood vessel.
Or:
More than one tumor has formed in the intrahepatic bile duct and may have spread into a blood vessel.
Cancer has spread through the wall of the perihilar bile duct to nearby fatty tissue or liver tissue.Stage 2 has two substages:
2A: Cancer has spread fewer than 5 mm into the wall of the distal bile duct and has spread to 1–3 nearby lymph nodes or 5–12 mm into the wall of the distal bile duct
2B: Cancer has spread 5 mm or more into the wall of the distal bile duct. Cancer may have spread to 1–3 nearby lymph nodes.
Symptoms are not likely to occur in these early stages.
3Stage 3 has two substages:
3A: Tumor has spread through the outer lining of the liver.
3B: Cancer has spread to organs or tissues near the liver, such as the:
• colon
• stomach
• duodenum
• diaphragm
• common bile duct
• abdominal wall
Or,
The cancer has spread to nearby lymph nodes.
Stage 3 has three substages:
3A: Cancer has spread to branches on one side of the hepatic artery or of the portal vein.
3B: Cancer has spread to other areas, such as the:
• common hepatic artery
• portal vein
• hepatic ducts
3C: Cancer has spread to 1–3 nearby lymph nodes.
Stage 3 has two substages:
3A: Cancer has spread into the wall of the distal bile duct and to 4 or more nearby lymph nodes.
3B: Cancer has spread to the large vessels that carry blood to the organs in the abdomen. It may also have spread to 1 or more nearby lymph nodes.
Symptoms may present at this stage as the tumor has grown in size and may block the bile duct. Symptoms may include:
• jaundice
• dark urine
• itchy skin
• abdominal pain
• nausea and vomiting
4Cancer has spread to most of the organs in the abdomen and distant parts of the body, such as:
• bones
• lungs
• distant lymph nodes
Stage 4 has two substages:
4A: Cancer has spread to 4 or more nearby lymph nodes.
4B: Cancer has spread to most of the organs in the abdomen and other parts of the body, which could include the:
• liver
• distant lymph nodes
• bones
• brain
• lungs
• skin
Cancer has spread to most of the organs in the abdomen and other parts of the body.
This may include the liver or lungs.
A person may experience both symptoms of bile duct cancer and other symptoms related to the area in which it has spread.

A person should talk with a doctor if they notice jaundice, yellowing of the eyes and skin. This can indicate that something is wrong with their liver.

However, jaundice can occur due to several different issues that affect the liver. As bile duct cancer is rare, jaundice is more likely to result from a liver problem such as hepatitis, cirrhosis, or alcohol-related liver disease.

It is difficult to detect bile duct cancer before it has spread enough to cause symptoms. There are no reliable screening tests to detect bile duct cancer early.

If a person has symptoms of a liver problem and a doctor rules out other, more common causes, they may search for the presence of bile duct cancer.

To diagnose bile duct cancer, a doctor will review both family and personal medical history. If they suspect cancer, they may order a number of tests, which may include one or more of the following:

Once doctors find bile duct cancer, it has usually progressed too far for surgical intervention. This makes the overall prognosis for bile duct cancer low.

Doctors often use 5-year survival rates to help give an idea of the person’s overall outlook. Doctors base these estimates on data available about the number of people still alive following a diagnosis of different stages of bile duct cancer.

While they can be helpful, it is important to note that they do not provide the full picture. A person’s overall health, age, and other factors can increase or decrease a person’s chance of survival.

For intrahepatic bile duct cancers, the following 5-year survival rates based on stage are:

  • Localized: 24%
  • Regional, or spread to nearby tissue or lymph nodes: 9%
  • Distant, or spread to far away organs: 2%
  • All stages combined: 9%

For extrahepatic bile duct cancers, the following 5-year survival rates based on stage are:

  • Localized: 17%
  • Regional, or spread to nearby tissue or lymph nodes: 16%
  • Distant, or spread to far away organs: 2%
  • All stages combined: 10%

The following sections provide answers to frequently asked questions about bile duct cancer.

Can you have bile duct cancer with no symptoms?

Yes, the early stages of bile duct cancer have no obvious signs or symptoms unless a tumor blocks a duct. Bile duct cancer may only start to display obvious symptoms in stages 3 or 4.

The most common sign of bile duct cancer is jaundice, but this can also be the result of several different conditions that affect the liver.

Would bile duct cancer show up in blood tests?

One of the issues with finding bile duct cancer early is that no reliable blood or visual tests can check for its presence.

During diagnosis, blood tests may help doctors determine the livers’ function and help them find tumor markers, but doctors will need to perform additional tests to detect bile duct cancer.

How do you know if bile duct cancer has spread?

A doctor will need to test for the presence of cancer in other areas of the body.

If the cancer has spread, a person may experience other signs and symptoms related to the location to which it has spread.

Bile duct cancer starts in the bile duct and may appear inside or outside the liver.

Bile duct cancer does not typically show signs or symptoms until it blocks a bile duct in its later stages. A person is unlikely to discover a tumor until they show symptoms of bile duct cancer and go in for an examination.

Diagnosis typically involves the use of several tests, including MRI and CT scans.