Disease: Cholangiocarcinoma (bile duct cancer)

    Overview

    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.

    Cholangiocarcinoma care at Mayo Clinic

    Source: http://www.mayoclinic.com

    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).

    Source: http://www.mayoclinic.com

    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.

    Source: http://www.mayoclinic.com

    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.

    Source: http://www.mayoclinic.com

    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.

    Source: http://www.mayoclinic.com

    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.

    Source: http://www.mayoclinic.com

    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.

    Source: http://www.mayoclinic.com

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