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

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).

Causes

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.

Risk factors

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.

Prevention

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.

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?
• Does anything improve or worsen your symptoms?

Overview

Hilar cholangiocarcinoma is a type of bile duct cancer that occurs in the bile ducts that lead out of the liver (hepatic ducts) and join with the gallbladder. Hilar cholangiocarcinomas are also known as Klatskin tumors.

Diagnosis

Tests and procedures used to diagnose hilar cholangiocarcinoma include:

• Imaging tests, such as computerized tomography (CT) and magnetic resonance imaging (MRI)
• Blood test for very high levels of the CA 19-9 tumor marker

• Biopsy using endoscopic retrograde cholangiopancreatography (ERCP) or endoscopic ultrasound and specialized lab testing with fluorescence in situ hybridization (FISH)

  Doctors typically don't recommend collecting cells using a needle inserted through your skin and into your bile duct (transperitoneal biopsy). This technique increases the risk of cancer recurrence and may make you ineligible for a liver transplant.

Treatment

Which treatment is best for you will depend on the location and extent of your hilar cholangiocarcinoma. Treatment typically involves surgery, liver transplant or supportive treatments to reduce your pain and other symptoms.

Surgery

Surgery for hilar cholangiocarcinoma involves removing:

• The cancer
• Surrounding bile ducts
• Portions of the liver
• Nearby lymph nodes

The surgeon then connects the remaining bile ducts to the small intestine so that bile can still reach your digestive tract and help digest food.

Liver transplant

Some people with early-stage hilar cholangiocarcinoma may consider liver transplant. Chemotherapy and radiation are typically used before the liver transplant to kill as many cancer cells as possible before surgery.

This treatment typically involves:

• Intravenous chemotherapy with a medicine that makes your cells more vulnerable to radiation
• External beam radiation therapy
• Internal radiation (brachytherapy) using small wires that are placed near the cancer and slowly release radiation
• Chemotherapy in pill form until transplant surgery
• Minimally invasive surgery to look for signs that cancer has spread
• Liver transplant with a donor liver if one becomes available

You may undergo routine tests to evaluate your health while waiting for a liver transplant to make sure you're healthy enough for the surgery.

Chemotherapy

People with advanced hilar cholangiocarcinoma who aren't able to undergo surgery or liver transplant may receive chemotherapy to slow the growth of the cancer.