Cholangiocarcinoma (Bile Duct Cancer)

Medically Reviewed by Jennifer Robinson, MD on March 18, 2024
6 min read

Bile duct cancer, also called cholangiocarcinoma, is when unusual cells grow out of control inside your bile ducts. Those are thin tubes about 4 to 5 inches long that move a fluid called bile from your liver to your gallbladder and small intestine. Bile helps you digest fat in the food you eat.

For some people, treatment can destroy the cancer. In others, it may never go away entirely. You may need regular doses of chemotherapy, radiation, or other treatments to keep it in check.

Learning more about the condition and getting support from your medical team and your loved ones can help you manage stress and concerns about the future.

Doctors identify bile duct cancer based on where it starts:

  • Outside your liver (extrahepatic). This type occurs more often and is more treatable. Cancer might form in one of two areas.
    • The hilum region, where your left and right bile ducts come together to form the common hepatic duct. This is called perihilar cancer.
    • The distal region, where your common bile duct passes through your pancreas. This is called distal cancer.
  • Inside your liver (intrahepatic). This type makes up 5% to 10% of cases.

Experts aren’t sure what causes bile duct cancer. Research shows that certain things can raise your chances of getting it, including long-term inflammation from conditions including:

Primary sclerosing cholangitis. This inflammation of your bile duct leads to scarring. Doctors don’t know what causes it. Many people who have it also have ulcerative colitis, an inflammation of the large intestine.

Bile duct stones. These are similar to gallstones but much smaller.

Choledochal cysts. Some people are born with a rare condition that causes bile-filled sacs along your bile ducts. Without treatment, they may lead to bile duct cancer.

Liver fluke infection. This is rare in the U.S. but more common in Asia. It happens when people eat raw or poorly cooked fish that’s infected with tiny parasitic worms called liver flukes. They can live in your bile ducts and cause cancer.

Reflux. When digestive juices from your pancreas flow back into your bile ducts, they can’t empty properly.

Cirrhosis. Alcohol and hepatitis can damage your liver and cause scar tissue, raising the risk of bile duct cancer.

Other things that can make you more likely to get bile duct cancer include:

Signs of bile duct cancer include:

Your doctor will start with a physical exam. They’ll ask about your general health, family history of cancer and liver disease, lifestyle, and habits, including drinking and smoking. They’ll also check for masses, tenderness, or fluid buildup in your belly.

Certain tests can find signs of bile duct cancer, including:

Blood tests. Some of these will tell your doctor whether your liver is working the way it should. Others look for signs of tumors, called markers. Your doctor may also check your levels of bilirubin, which can cause jaundice when you have a lot of it.

Abdominal ultrasound. This imaging test helps your doctor look for tumors.

CT scan or MRI. A CT scan is a powerful X-ray that makes detailed pictures inside your body. An MRI uses high-powered magnets to show organs and other things inside your body. If you have a tumor, these can show its size and location, as well as how healthy your liver is.

Endoscopy. This uses a tool called an endoscope that’s like a camera on the end of a cable. It lets your doctor see inside your body without surgery. They can look at your esophagus, your stomach, and the beginning of your lower intestine.

Cholangioscopy. This procedure helps check your bile ducts for problems. In ERCP, or endoscopic retrograde cholangiopancreatography, your doctor uses an endoscope to inject dye into your bile ducts. They then take X-rays to look for tumors.

Magnetic resonance cholangiopancreatography (MRCP). This uses an MRI machine to make images of your bile ducts.

Percutaneous transhepatic cholangiography (PTC). Your doctor inserts a needle through your skin and into a bile duct in your liver. They inject dye and take an X-ray of the area.

Laparoscopy. Your doctor uses medication to put you to sleep and then makes a small cut in your belly. They insert a thin tube with a light and a video camera to look at your bile duct, gallbladder, liver, and other organs and tissues.

Biopsy. Your doctor takes a sample of bile duct cells or tissue, and a technician checks them under a microscope. They might do this during a laparoscopy.

You might have one or more of these treatments:

Biliary drainage. If your bile duct is blocked, your doctor may do a bypass, cutting it off and reattaching it on the other side of the blockage. They could also put a tube called a stent into the duct to keep bile flowing.

Photodynamic therapy. Your doctor injects an inactive form of a certain medication into your vein. The drug tends to collect in more cancer cells than healthy cells. After a few days, they use an endoscope to aim a special light at the tumor. It activates the drug, killing the cancer cells.

Radiation. This uses high-energy rays or particles to kill cancer cells. Your doctor may use it before surgery to shrink a tumor. After surgery, it can kill any cancer cells that remain. If your doctor can’t remove the cancer but it hasn’t spread, radiation can help keep it under control.

Chemotherapy. As with radiation, doctors often use medicines to kill cancer cells before or after surgery. You can take chemo by mouth or through a shot into a vein.

Surgery. Depending on the cancer’s spread, your doctor might need to remove some or all of your bile duct, lymph nodes, liver, pancreas, or small intestine.

Liver transplant. This is a rare treatment that can sometimes cure bile duct cancer. Your doctor might use chemo and radiation while you wait for a new liver.

The success of your treatment will depend on many things, including where the cancer is and how far along it is when you’re diagnosed. Your bile duct is deep inside your body, so unlike with some other cancers, you won’t see or feel problems in the early stages.

People whose cancers start outside the liver tend to do better than those who have tumors inside it. But if the cancer has spread to other parts of your body, the outcome is about the same no matter where it starts.

Even during cancer treatment, you can take steps to feel as healthy as possible. Start by cutting down on alcohol and quitting tobacco.

Cancer often causes fatigue or extreme tiredness. You might be so tired that it’s hard to do the things you want to do. Rest will help, but so will some exercise. Talk with your doctor about a program that’s right for you.

When you learn that you have cancer, you’ll most likely wonder about your future. This can be a stressful time, so seek support where you can. Talk to someone you trust: a family member or friend, a counselor, or a religious leader.

For more information and support, visit the American Cancer Society at www.cancer.org.