Learn about symptoms, risk factors, diagnosis and treatment, including transplant, for this rare cancer that affects the bile ducts of older adults.
Cholangiocarcinoma is a type of 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.
Cholangiocarcinoma, also known as bile duct cancer, 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 often diagnosed when it's advanced, making successful treatment difficult to achieve.
The gallbladder stores bile, a yellow-green fluid produced in the liver. Bile flows from your liver into your gallbladder, where it's held until needed during the digestion of food. When you eat, your gallbladder releases bile into the bile duct, where it's carried to the upper part of the small intestine, called the duodenum, to help break down fat in food.
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 happens when cells in the bile ducts develop changes in their DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cells to multiply out of control and form a mass of cells (tumor) that can invade and destroy healthy body tissue. It's not clear what causes the changes that lead to cholangiocarcinoma.
Factors that may increase your risk of cholangiocarcinoma include:
To reduce your risk of cholangiocarcinoma, you can:
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 you choose to drink. For healthy adults, that means up to one drink a day for women and up to two drinks a day for men. Maintain a healthy weight. When working with chemicals, follow the safety instructions.
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 carbohydrate 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.
Endoscopic retrograde cholangiopancreatography (ERCP) uses a dye to highlight the bile ducts and pancreatic duct on X-ray images. A thin, flexible tube with a camera on the end, called an endoscope, is passed down your throat and into your small intestine. The dye enters the ducts through a small hollow tube, called a catheter, passed through the endoscope. Tiny tools passed through the catheter also can be used to remove gallstones.
Treatments for cholangiocarcinoma (bile duct cancer) may include:
Because cholangiocarcinoma is a very difficult type of cancer 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 new drugs 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 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.
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.
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:
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.
Start by making an appointment with your doctor if you have any signs or symptoms that worry you. If you doctor determines that you have cholangiocarcinoma, he or she may refer you to a doctor who specializes in diseases of the digestive system (gastroenterologist) or to a doctor who specializes in treating cancer (oncologist).
Some basic questions to ask your doctor include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask additional questions during your appointment.
Your doctor is likely to ask you a number of questions, such as: