Symptoms

Signs and symptoms of cholangiocarcinoma include:

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

Diagnosis

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.

Bile Duct Cancer Treatment

The results of your imaging tests will help guide your Bile Duct Cancer treatment.

Bile Duct Cancer treatment for cholangiocarcinoma may include:

• 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 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

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.