Bile duct cancer is cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile. Bile ducts connect your liver to your gallbladder and to your small intestine.

Bile duct cancer, also known as cholangiocarcinoma, is a rare form of cancer that occurs mostly in people older than age 50, though it can occur at any age.

Doctors divide bile duct cancer into different types based on where the cancer occurs in the bile ducts:

  • Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver and is sometimes classified as a type of liver cancer.
  • Hilar cholangiocarcinoma occurs in the bile ducts just outside of the liver. This type is also called perihilar cholangiocarcinoma.
  • Distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine

Bile duct cancer is an aggressive form of cancer that progresses quickly and is difficult to treat.

Signs and symptoms of bile duct cancer include:

  • Yellowing of the skin and eyes (jaundice)
  • Intensely itchy skin
  • White-colored stools
  • Fatigue
  • Abdominal pain
  • Unexplained weight loss

When to see a doctor

Make an appointment with your doctor if you have any persistent signs and symptoms that worry you.

It's not clear what causes most cases of bile duct cancer.

Bile duct cancer occurs when cells in the bile ducts develop changes (mutations) in their DNA — the material that provides instructions for every chemical process in your body. DNA mutations cause changes in the instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of cancerous cells.

Factors thought to increase the risk of bile duct cancer include:

  • Primary sclerosing cholangitis. This disease causes hardening and scarring of the bile ducts.
  • Chronic liver disease. Scarring of the liver caused by a history of chronic liver disease increases the risk of bile duct cancer.
  • Bile duct problems present at birth. People born with a choledochal cyst, which causes dilated and irregular bile ducts, have an increased risk of bile duct cancer. Most cases of choledochal cysts are diagnosed in childhood, though some people are diagnosed as adults.
  • A liver parasite. In areas of Southeast Asia, bile duct cancer is associated with liver fluke infection, which can occur from eating raw or undercooked fish.
  • Older age. Bile duct cancer occurs most often in older adults.
  • Smoking. Smoking is associated with an increased risk of bile duct cancer.

Tests and procedures used to diagnose bile duct cancer include:

  • Liver function tests. Blood tests to measure your liver function can give your doctor clues about what's causing your signs and symptoms.
  • Tumor marker test. Checking the level of cancer antigen (CA) 19-9 in your blood may give your doctor additional clues about your diagnosis. CA 19-9 is a protein that's overproduced by bile duct cancer cells.

    A high level of CA 19-9 in your blood doesn't mean you have bile duct cancer, though. This result can also occur in other bile duct diseases, such as bile duct inflammation and obstruction.

  • Imaging tests. Imaging tests, such as an MRI with a special dye (contrast) to highlight your bile ducts, can help your doctor see any abnormalities that may indicate bile duct cancer.
  • A test to examine your bile duct with a small camera. During endoscopic retrograde cholangiopancreatography (ERCP), a thin tube equipped with a tiny camera is passed down your throat and through your digestive tract to your small intestine.

    During ERCP, the camera is used to examine the area where your bile ducts connect to your small intestine. Your doctor may also use this procedure to inject dye into the bile ducts to help them show up better on imaging tests.

  • A procedure to remove a sample of tissue for testing. A biopsy procedure involves collecting a sample of suspicious tissue for testing in a laboratory. How the sample is collected depends on which part of the bile duct is affected.

    If the suspicious area is located very near where the bile duct joins the small intestine, a biopsy sample may be obtained during ERCP. If the suspicious area is within or near the liver, your doctor may recommend removing a tissue sample using a long needle. An imaging test, such as an ultrasound or CT, is used to guide the needle to the precise area.

Once your doctor determines that you have bile duct cancer, he or she will work to understand the extent (stage) of the cancer. Often this involves additional imaging tests. Your cancer's stage helps determine your prognosis and your treatment options.

Treatments for bile duct cancer may include:

  • Surgery. When possible, doctors try to remove as much of the cancer as they can. For very small bile duct cancers, this involves removing part of the bile duct and joining the cut ends. For more-advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes may be removed as well.
  • Liver transplant. Surgery to remove your liver and replace it with one from a donor (liver transplant) may be an option in certain cases for people with hilar cholangiocarcinoma. For many, a liver transplant is a cure for hilar cholangiocarcinoma, but there is a risk that bile duct cancer will recur after a liver transplant.
  • Chemotherapy. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be used before a liver transplant. It may also be an option for people with advanced bile duct cancers to help slow the disease and relieve signs and symptoms.
  • Radiation therapy. Radiation therapy uses high-energy sources, such as X-rays and protons, to damage or destroy cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body (external beam radiation), or it can involve placing radioactive material inside your body near the site of your cancer (brachytherapy).
  • Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the fast-growing cancer cells. Laser light directed at the cancer causes a chemical reaction in the cancer cells, killing them. This treatment can help relieve signs and symptoms, and may also slow cancer growth.
  • Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. Biliary drainage can involve bypass surgery to reroute the bile around the cancer or stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of bile duct cancer.

If you have signs and symptoms that worry you, start by seeing your family doctor. If your doctor suspects you may have bile duct cancer, you may be referred to a doctor who specializes in diseases of the digestive tract (gastroenterologist) or to a doctor who specializes in treating cancer (oncologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared. Here's some information to help you get ready and what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, as well as any vitamins or supplements, that you're taking.
  • Consider taking a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.
  • Bring copies of your medical records to your appointment, if you're seeing a new doctor for the first time. If you've had scans done at another facility, ask that files containing those images be placed on a CD and bring that to your appointment.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For bile duct cancer, some basic questions to ask your doctor include:

  • What type of bile duct cancer do I have?
  • What is the stage of my bile duct cancer?
  • What does my pathology report say? Can I have a copy of the pathology report?
  • Will I need more tests?
  • What are my treatment options?
  • What are the potential side effects of each treatment option?
  • Is there one treatment you recommend over the others?
  • How will my treatment affect my daily life?
  • How much time can I take to make my decision about bile duct cancer treatment?
  • Should I seek a second opinion?
  • Should I see a bile duct cancer specialist? What will that cost, and will my insurance cover it?
  • Are there brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask additional questions during your appointment.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may allow more time later to cover other points you want to address. Your doctor may ask:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?

Learning that you have any life-threatening illness can be devastating. Each person finds his or her own ways of coping with a diagnosis of bile duct cancer. Although there are no easy answers for people dealing with cancer, the following suggestions may be of help:

  • Learn enough about bile duct cancer to make decisions about your care. Ask your doctor about your bile duct cancer, including the type and stage of your cancer, your treatment options and, if you like, your prognosis. As you learn more about bile duct cancer, you may become more confident in making treatment decisions.
  • Keep friends and family close. Keeping your close relationships strong will help you deal with your bile duct cancer. Friends and family can provide the practical support you'll need, such as helping take care of your house if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by cancer.
  • Find someone to talk with. Find a good listener with whom you can talk about your hopes and fears. This may be a friend or family member. The support of a counselor, medical social worker, clergy member or cancer survivors' group also may be helpful.

    Ask your doctor about support groups in your area. Or check your phone book, library or a cancer organization, such as the National Cancer Institute or the American Cancer Society.

  • Make plans for the unknown. Having a life-threatening illness, such as cancer, requires you to prepare for the possibility that you may die. For some people, having a strong faith or a sense of something greater than themselves makes it easier to come to terms with a life-threatening illness.

Ask your doctor about advance directives and living wills to help you plan for end-of-life care, should you need it.

Bile duct cancer is rare and there's no certain way to prevent it. However, you may reduce your risk of the disease if you:

  • Stop smoking. Smoking is linked to an increased risk of bile duct cancer. If you smoke, stop. If you've tried quitting in the past and haven't been successful, talk with your doctor about strategies to help you quit.

  • Reduce your risk of liver disease. Chronic liver disease is associated with an increased risk of bile duct cancer. Some causes of liver disease can't be prevented, but others can. Do what you can to take care of your liver.

    For instance, to reduce your risk of liver inflammation (cirrhosis), drink alcohol in moderation, if at all. Maintain a healthy weight. When working with chemicals, follow the safety instructions on the container.

Last Updated: 2016-07-09
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