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Cholangiocarcinoma 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. This condition, also known as bile duct cancer, is an uncommon form of cancer that occurs mostly in people older than age 50, though it can occur at any age.
Doctors divide cholangiocarcinoma 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.
Cholangiocarcinoma is a type of tumor that is very difficult to treat.
Signs and symptoms of cholangiocarcinoma include:
Yellowing of your skin and the whites of your eyes (jaundice)
Intensely itchy skin
Unintended weight loss
When to see a doctor
See your doctor if you have persistent fatigue, abdominal pain, jaundice, or other signs and symptoms that bother you. He or she may refer you to a specialist in digestive diseases (gastroenterologist).
Cholangiocarcinoma 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. It's not clear what causes the genetic mutations that lead to cancer.
Factors that may increase your risk of cholangiocarcinoma 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 cholangiocarcinoma.
Bile duct problems present at birth. People born with a choledochal cyst, which causes dilated and irregular bile ducts, have an increased risk of cholangiocarcinoma.
A liver parasite. In areas of Southeast Asia, cholangiocarcinoma is associated with liver fluke infection, which can occur from eating raw or undercooked fish.
Older age. Cholangiocarcinoma occurs most often in adults over age 50.
Smoking. Smoking is associated with an increased risk of cholangiocarcinoma.
There's no way to prevent cholangiocarcinoma (bile duct cancer). But you may reduce your risk of the disease if you:
Stop smoking. Smoking is linked to an increased risk of cholangiocarcinoma. 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 cholangiocarcinoma. 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.
A study published in 2016 showed that aspirin use may help reduce the risk of developing cholangiocarcinoma. The study involved data on nearly 4,800 people. Further study is needed to be certain that long-term aspirin use is safe for cancer prevention.
If your doctor suspects cholangiocarcinoma, he or she may have you undergo one or more of the following tests:
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.
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. 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.
Imaging tests. Imaging tests can help your doctor see any abnormalities in your internal organs that may indicate cholangiocarcinoma. Techniques used to diagnose bile duct cancer include computerized tomography (CT) scans and magnetic resonance imaging (MRI) combined with magnetic resonance cholangiopancreatography (MRCP). MRCP is increasingly being used as a noninvasive alternative to ERCP. It offers 3-D images without the need for a dye to enhance the images.
A procedure to remove a sample of tissue for testing. A biopsy is a procedure to remove a small sample of tissue for examination under a microscope.
If the suspicious area is located very near where the bile duct joins the small intestine, your doctor may obtain a biopsy sample during ERCP. If the suspicious area is within or near the liver, your doctor may obtain a tissue sample by inserting a long needle through your skin to the affected area (fine-needle aspiration). He or she may use an imaging test, such as an endoscopic ultrasound or CT scan, to guide the needle to the precise area.
How your doctor collects a biopsy sample may influence which treatment options are available to you later. For example, if your bile duct cancer is biopsied by fine-needle aspiration, you will become ineligible for liver transplantation. Don't hesitate to ask about your doctor's experience with diagnosing cholangiocarcinoma. If you have any doubts, get a second opinion.
If your doctor confirms a diagnosis of cholangiocarcinoma, he or she tries to determine 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 cholangiocarcinoma (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 the 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 cholangiocarcinoma to help slow the disease and relieve signs and symptoms.
Radiation therapy. Radiation therapy uses high-energy sources, such as photons (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. You'll typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. You'll need to avoid sun exposure after treatments.
Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. It 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 cholangiocarcinoma.
Because cholangiocarcinoma is a very difficult type of tumor to treat, don't hesitate to ask about your doctor's experience with treating the condition. If you have any doubts, get a second opinion.
Clinical trials are studies to test new treatments, such as systemic therapy and new approaches to surgery. If the treatment being studied proves to be safer and more effective than are current treatments, it can become the new standard of care.
Clinical trials for cholangiocarcinoma might give you a chance to try new targeted therapy, or chemotherapy drugs.
Clinical trials can't guarantee a cure, and they might have serious or unexpected side effects. On the other hand, cancer clinical trials are closely monitored to ensure they're conducted as safely as possible. They offer access to treatments that wouldn't otherwise be available to you.
Talk to your doctor about what clinical trials might be appropriate for you.
Supportive (palliative) care
Palliative care is specialized medical care that focuses on providing relief from pain and other symptoms of a serious illness. Palliative care specialists work with you, your family and your other doctors to provide an extra layer of support that complements your ongoing care. Palliative care can be used while undergoing aggressive treatments, such as surgery.
When palliative care is used along with other appropriate treatments — even soon after your diagnosis — people with cancer may feel better and may live longer.
Palliative care is provided by teams of doctors, nurses and other specially trained professionals. These teams aim to improve the quality of life for people with cancer and their families. Palliative care is not the same as hospice care or end-of-life care.
Coping and support
Learning you have a life-threatening illness can be devastating. You will not find any easy answers for dealing with cholangiocarcinoma, but some of the following suggestions may help:
Learn what you need to know about your cancer. Ask your doctor about your cancer, including the type and stage of your cancer, your treatment options and, if you like, your prognosis. As you learn more about cholangiocarcinoma, you may become more confident in making treatment decisions. Ask about trusted sources of further information.
If you're doing your own research, good places to start include the National Cancer Institute.
Keep friends and family close. Keeping your close relationships strong will help you deal with your 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.
Find someone to talk with. Although friends and family can be your best allies, in some cases they have difficulty coping with the shock of your diagnosis. In these cases, talking with a counselor, medical social worker, or a pastoral or religious counselor can be helpful. Ask your doctor for a referral.
Connect with other cancer survivors. You may find comfort in talking with other cancer survivors. Contact your local chapter of the American Cancer Society to find cancer support groups in your area.
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.
Preparing for an appointment
Start by making an appointment with your doctor if you have any signs or symptoms that worry you. He or she may recommend tests and procedures to investigate your signs and symptoms. If you doctor determines that you have cholangiocarcinoma, he or she may refer you to a doctor who specializes in diseases of the digestive tract (gastroenterologist) or to a doctor who specializes in treating cancer (oncologist).
What you can do
Be aware of any pre-appointment restrictions, such as restricting your diet.
List your symptoms, including any that seem unrelated to the reason you scheduled the appointment.
List key personal information, including recent changes or stressors.
List all of your medications, vitamins and supplements, including doses.
Ask a relative or friend to accompany you, to help you remember what the doctor says.
List 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.
Some basic questions to ask your doctor include:
Do I have bile duct cancer? What type?
What is the stage of my 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 think is best for me?
How will my treatment affect my daily life?
How much time can I take to make my decision about bile duct cancer treatment?
What is your experience with bile duct cancer diagnosis and treatment? How many surgical procedures for this type of cancer are done each year at this medical center?
Should I see a bile duct cancer specialist? What will that cost, and will my insurance cover it?
Do you have any 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, such as:
When did you first begin experiencing symptoms?
How severe are your symptoms? Are they occasional or continuous?