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:
Cholangiocarcinoma is a type of tumor that is very difficult to treat.
Cholangiocarcinoma care at Mayo Clinic
Signs and symptoms of cholangiocarcinoma include:
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 â 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 â a mass of cancerous cells. It's not clear what causes the genetic mutations that lead to cancer.
If your doctor suspects cholangiocarcinoma, he or she may have you undergo one or more of the following tests:
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 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.
There's no way to prevent cholangiocarcinoma (bile duct cancer). But you may reduce your risk of the disease if you:
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.
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.
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.
Factors that may increase your risk of cholangiocarcinoma include: