Bile duct cancer, or cholangiocarcinoma, is a type of cancer that forms in the thin tubes, or bile ducts, that carry the digestive fluid bile from your liver to the gallbladder and small intestine. This is an uncommon type of cancer that occurs most often in people over fifty, although it can happen at any age. There are three main types of cholangiocarcinoma. The types represent where the cancer is growing in the bile ducts. All these types of bile duct cancer are difficult to treat. The three types are:

  • Intrahepatic cholangiocarcinoma happens in the part of the bile duct within the liver and is classified as a type of liver cancer.
  • Hilar cholangiocarcinoma, or perihilar cholangiocarcinoma, occurs in the bile ducts just outside of the liver.
  • Distal cholangiocarcinoma occurs in the bile duct nearest to the small intestine.

What are the causes and risk factors for bile duct cancer?

This type of cancer occurs in the body when the cells in the bile duct begin to change due to DNA damage or mutation and have abnormal constant growth. Eventually, these abnormal cells can grow into a tumor, which could be cancerous. It is not entirely clear on what triggers this abnormal cell growth, although certain risk factors have been established. These factors may include:

  • Primary sclerosing cholangitis is a disease that causes hardening and scarring of the bile ducts.
  • Chronic liver disease causes scarring of the liver caused by a history of chronic liver disease increases the risk of cholangiocarcinoma.
  • Bile duct problems present at birth occur in people born with a choledochal cyst, which causes dilated and irregular bile ducts, and an increased risk of cholangiocarcinoma.
  • Liver parasites happen in areas of Southeast Asia, and cholangiocarcinoma is associated with liver fluke infection, which can occur from eating raw or undercooked fish.
  • Older age. Raises the risk of cholangiocarcinoma, as this cancer occurs most often in adults over age fifty.
  • Smoking is associated with an increased risk of cholangiocarcinoma.

What are the symptoms to watch for with this type of cancer?

The signs to watch for with cholangiocarcinoma’s are:

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

If you are experiencing extreme fatigue, abdominal pain, jaundice or any other bothersome symptoms, you should make an appointment with your doctor. Early diagnosis and treatment is key, and from this point, you can then be referred to a gastroenterologist which is a specialist in digestive diseases.

Are there any ways to reduce or prevent this type of cholangiocarcinoma?

No, there is no true way to prevent this type of cancer. You may reduce your risk by quitting your use of tobacco products if you are a smoker or user. If you have tried quitting in the past and failed, consider talking with your doctor about the newest medications designed to help you quit and nix the cravings. Doing what you can to keep your liver healthy and free of disease can also help lower your risk for bile duct cancer. This means not drinking alcohol in excess, reducing inflammation in the liver, and maintaining a healthy and constant weight. If you have a job where heavy chemicals are involved, always wear all proper precautionary protective gear and follow directions for each specific toxin.

What tests are done to diagnose bile duct cancer?

After your examination and discussion with your doctor, they may order one or more of the following tests and procedures:

  • Liver function tests are blood tests to measure your liver function that can give your doctor clues about what is causing your signs and symptoms.
  • Tumor marker test is done to check the level of cancer antigen 19-9 in your blood may give your doctor additional clues about your diagnosis. CA 19-9 is a protein that is overproduced by bile duct cancer cells.
  • A high level of CA 19-9 in your blood does not 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 can be done during an endoscopic retrograde cholangiopancreatography, or ERCP, which is a thin tube equipped with a tiny camera. This tube 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 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, or CT, scans and magnetic resonance imaging, or MRIs, combined with magnetic resonance cholangiopancreatography, which is also called 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 is a biopsy which 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 during a 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.

Once your doctor determines the stage of your bile duct cancer, additional imaging tests are almost always needed. The stage of your cancer will help to determine which treatment plan best fits your needs.

What are the most common treatments for bile duct cancer?

There are numerous treatments and combinations of treatments that your doctors and specialists may use during your care. These treatments may be:

  • Whenever 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 the more advanced bile duct cancers, nearby liver tissue, pancreas tissue or the lymph nodes may be removed as well.
  • Liver transplant. A transplant may be done to replace your liver with one from a donor. This may only 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 uses high-energy sources like photons and protons to damage or destroy cancer cells. Radiation therapy can involve a machine that directs radiation beams at your body. Or it can involve placing radioactive material inside your body near the site of your cancer, which is called brachytherapy.
  • Photodynamic therapy is a light-sensitive chemical that is injected into a vein and accumulates in the fast-growing cancer cells. Laser light is directed at the cancer and causes a chemical reaction in the cancer cells, killing them. One would typically need multiple treatments. Photodynamic therapy can help relieve your signs and symptoms, and it may also slow cancer growth. Avoiding sun exposure after treatments is key.
  • 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.

Due to the difficulty in treating these types of cancer, never hesitate to talk to your doctor about your doctor or specialists’ history and experience with this condition.

What can I expect from my first appointment? Certain questions I should ask?

After you have made your appointment, write down any questions or concerns you have before meeting with your doctor. From there they will examine you and order several tests to determine and investigate the signs and symptoms you have been experiencing. If it is diagnosed that you have bile duct cancer, you may be referred to an oncologist or a gastroenterologist- or both!

To be the best patient you can to help reclaim your health and body, consider taking these things with you to your appointments:

  • Be aware of any pre-appointment restrictions, such as restricting your diet or avoiding certain food dyes on imaging days.
  • List your symptoms, including any that seem unrelated to the reason you scheduled the appointment.
  • List key personal information, including recent changes or stressors seriously affecting your life.
  • List all your medications, vitamins, and supplements, including doses taken daily.
  • Ask a relative or friend to accompany you, to help you remember what the doctor says and take notes if needed.
  • List questions to ask your doctor.
  • Bring copies of your medical records to your appointment, if you are seeing a new doctor for the first time. If you have had scans or imaging done at another facility, ask that files containing those images be placed on a CD or printed and bring that to your appointment.

Here are some basic questions you may want to ask your doctor while at your appointment, or at future appointments. They are:

  • 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?

Any cancer diagnosis can be a very scary and life-altering event. Educating yourself about your condition and having an open and honest relationship with your medical care team will ensure you are on the best track possible for whole healing.

Citations:

American Cancer Society

National Institutes of Health and Human Services

Mayo Clinic