Description

Cholangiocarcinoma is a type of cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile. Bile ducts connect the liver to the gallbladder and small intestine.

Cholangiocarcinoma, also known as bile duct cancer, occurs mainly in people older than 50 years, although 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 in the liver and is sometimes classified as a type of liver cancer.
  • Hilar cholangiocarcinoma occurs in the bile ducts outside of the liver. This type is also called perihilar cholangiocarcinoma.
  • Distal cholangiocarcinoma occurs in the portion of the bile ducts closer in the small intestine. This type is also called extrahepatic cholangiocarcinoma.

Cholangiocarcinoma is often diagnosed when it is advanced, making the success of the treatment difficult to achieve.

Symptoms

The signs and symptoms of cholangiocarcinoma include:

  • Yellowing of the skin and the whites of the eyes (jaundice)
  • The intense itching of the skin
  • White color of the clay
  • Fatigue
  • Abdominal pain in the right side, just below the ribs
  • Losing weight without trying
  • Fever
  • Night sweats
  • Dark colored urine

When to see a doctor

Consult your doctor if you have persistent fatigue, abdominal pain, jaundice or other signs and symptoms that worry you. He or she may refer you to a specialist in diseases of the digestive system (gastroenterologist).

Causes

Cholangiocarcinoma occurs when the 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 that tell the cells to multiply out of control and form a mass of cells (tumor) that can invade and destroy healthy body tissue. It is not clear what causes the changes that lead to cholangiocarcinoma.

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 disease of the liver. The scarring of the liver caused by a history of chronic liver disease increases the risk of cholangiocarcinoma.
  • Bile duct problems present at birth. People who are born with a choledochal cyst, which causes dilatation and irregular bile ducts, have an increased risk of cholangiocarcinoma.
  • A parasite of the liver. In areas of Southeast Asia, cholangiocarcinoma is associated with liver fluke infection, which can occur by eating raw or undercooked fish.
  • Advanced age. Cholangiocarcinoma occurs most often in adults over 50 years of age.
  • The habit of smoking. Smoking is associated with an increased risk of cholangiocarcinoma.
  • Diabetes. The people who have type 1 or type 2 diabetes, may have an increased risk of cholangiocarcinoma.
  • Certain inherited conditions. Some changes in the DNA passed on from parents to children because of the conditions that increase the risk of cholangiocarcinoma. Examples of these conditions include cystic fibrosis, and the Lynch syndrome.

Prevention

To reduce the risk of cholangiocarcinoma, you can:

  • Stop smoking. Smoking is linked to an increased risk of cholangiocarcinoma. If you smoke, stop. If you have tried to quit in the past and I have not been successful, talk with your doctor about strategies to help you quit smoking.
  • Reduce your risk of liver disease.Chronic liver disease is associated with an increased risk of cholangiocarcinoma. Some of the causes of liver disease cannot be prevented, but others may. Do what you can to take care of your liver. For example, to reduce the risk of inflammation of the liver (cirrhosis), drinking alcohol in moderation, if you choose to drink. For healthy adults, that means up to one drink per day for women and up to two drinks per day for men. Maintain a healthy weight. When working with chemicals, always follow the safety instructions.

Reduce your risk of liver disease. Chronic liver disease is associated with an increased risk of cholangiocarcinoma. Some of the causes of liver disease cannot be prevented, but others may. Do what you can to take care of your liver.

For example, to reduce the risk of inflammation of the liver (cirrhosis), drinking alcohol in moderation, if you choose to drink. For healthy adults, that means up to one drink per day for women and up to two drinks per day for men. Maintain a healthy weight. When working with chemicals, always follow the safety instructions.

Diagnosis

If your doctor suspects that the cholangiocarcinoma, he or she may have you undergo one or more of the following tests:

  • The liver function tests. Blood tests to measure the function of your liver can give your doctor clues about what the cause of your signs and symptoms.
  • Tumor marker test.Check the level of carbohydrate antigen (CA) 19-9 in the blood can give your doctor additional clues about your diagnosis.CA19-9 is a protein that is produced in excess by the bile ducts cancer cells. A high level of ofCA19-9 in blood does not mean that you have cancer of the bile duct, however. This result can also occur in other bile duct diseases, such as the bile duct, inflammation and obstruction.
  • A test to examine your bile duct with a small camera. During endoscopic retrograde cholangiopancreatography (ERCP), a thin, flexible tube equipped with a tiny camera is passed down your throat and through your digestive tract to the small intestine. The camera is used to examine the area where the bile ducts connect to your small intestine. Your doctor can also use this procedure to inject dye into the bile ducts to help them look better on the test image.
  • Imaging tests. Imaging tests can help your doctor see your internal organs and look for signs of cholangiocarcinoma. The techniques used to diagnose bile duct cancer include ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) combined with magnetic resonance cholangiopancreatography (MRCP). MRCP is used increasingly as a noninvasive alternative to ERCP . Offers 3D images without the need of a contrast medium to enhance the images.
  • A procedure to remove a sample of tissue for analysis.A biopsy is a procedure to remove a small sample of tissue for examination under a microscope. If the suspicious area is located very close to where the bile duct joins the small intestine, the doctor can obtain a biopsy sample duringERCP. If the suspicious area is within or close to the liver, your doctor may obtain a tissue sample by inserting a long needle through the skin in the affected area (fine needle aspiration). He or she may use an imaging test, such as an endoscopic ultrasound orCTscan, to guide the needle to the precise area. How your doctor removes a sample of the biopsy may influence the treatment options are available to you later. For example, if the bile duct cancer is a biopsy by fine needle aspiration, you will be eligible for a liver transplant. Do not hesitate to ask your doctor about the experience with the diagnosis of cholangiocarcinoma. If you have any doubt, seek a second opinion.

Tumor marker test. Check the level of carbohydrate antigen (CA) 19-9 in the blood can give your doctor additional clues about your diagnosis. CA 19-9 is a protein that is produced in excess by the bile ducts cancer cells.

A high level of CA 19-9 in the blood does not mean that you have cancer of the bile duct, however. This result can also occur in other bile duct diseases, such as the bile duct, inflammation and obstruction.

A procedure to remove a sample of tissue for analysis. A biopsy is a procedure to remove a small sample of tissue for examination under a microscope.

If the suspicious area is located very close to where the bile duct joins the small intestine, the doctor can get a sample for biopsy during ERCP . If the suspicious area is within or close to the liver, your doctor may obtain a tissue sample by inserting a long needle through the skin in 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 removes a sample of the biopsy may influence the treatment options are available to you later. For example, if the bile duct cancer is a biopsy by fine needle aspiration, you will be eligible for a liver transplant. Do not hesitate to ask your doctor about the experience with the diagnosis of cholangiocarcinoma. If you have any doubt, seek a second opinion.

If the doctor confirms the diagnosis of cholangiocarcinoma, he or she tries to determine the extent (stage) of the cancer. This often involves additional imaging tests. Your cancer's stage helps determine the prognosis and treatment options.

Treatment

Treatments for cholangiocarcinoma (bile duct cancer) may include:

  • Surgery. When possible, surgeons try to remove as much of the cancer as possible. For very small bile duct cancer, this involves the removal of part of the bile duct and join the cut ends. For more advanced bile duct cancer, near liver tissue, pancreas tissue or lymph nodes can be removed.
  • Liver transplant. The surgery to remove the liver and replace it with one from a donor (liver transplant) may be an option in certain situations for people with hilar cholangiocarcinoma. For many, a liver transplant may be a cure for hilar cholangiocarcinoma, but there is a risk that the cancer will recur after liver transplantation.
  • Chemotherapy. Chemotherapy uses drugs to destroy cancer cells. Chemotherapy can be used before a liver transplant. It can also be an option for people with advanced cholangiocarcinoma, to help to slow the disease and alleviate the signs and symptoms. Chemotherapy drugs can be injected into a vein to that run throughout the body. Or the drugs can be administered in such a way that it is delivered directly to the cancer cells.
  • The radiation therapy. Radiation therapy uses high-powered energy beams from sources such as X-rays and protons, to kill cancer cells. The radiation therapy may involve a machine that directs the beam of radiation in your body (external radiation therapy). Or it can involve the placement of radioactive material inside your body near the site of the cancer (brachytherapy).
  • Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Your doctor may examine your cancer cells to see if the targeted therapy can be effective against your cholangiocarcinoma.
  • Immunotherapy. Immunotherapy uses your immune system to fight cancer. From your body to fight off diseases immune system will not attack the cancer because the cancer cells produce proteins that help to hide from the immune system cells. Immunotherapy works by interfering with that process. For cholangiocarcinoma, immunotherapy could be an option for advanced cancer when other treatments have not helped.
  • The heating of cancer cells. Radiofrequency ablation uses electric current to heat and destroy cancer cells. The use of a test image as a guide, as the ultrasound, the doctor inserts one or more thin needles into small incisions in the abdomen. When the needles reach the cancer, which is heated with an electric current, the destruction of cancer cells.
  • Photodynamic therapy. In photodynamic therapy, a light-sensitive chemical is injected into a vein and accumulates in the rapid growth of cancer cells. Laser light directed at the cancer, it causes a chemical reaction in the cancer cells, killing them. You usually need several treatments. Photodynamic therapy can help alleviate the signs and symptoms, and can also reduce the growth of cancer. You will need to avoid sun exposure after the treatment.
  • Biliary drainage. Biliary drainage is a procedure to restore the flow of bile. This may involve the placement of a thin tube into the bile duct to drain bile. Other strategies include bypass surgery to divert the bile around the cancer and the use of stents to hold open a bile duct is collapsed by the cancer. Biliary drainage helps to relieve the signs and symptoms of cholangiocarcinoma.

Because cholangiocarcinoma is a very difficult type of cancer to treat, do not hesitate to ask your doctor about the experience with the treatment of the condition. If you have any doubt, seek a second opinion.

Clinical trials

Clinical trials are studies to test new treatments, such as new drugs and new approaches to surgery. If the study treatment is more safe and more effective than current treatments, may become the new standard of care.

Clinical trials do not and cannot guarantee a cure, and that could have serious or unexpected side effects. On the other hand, the cancer clinical trials are closely monitored to ensure that it is performed as safely as possible. Offer access to treatments that are not to be available again for you.

Talk with your doctor about what clinical trials may be appropriate for you.

Supportive (palliative) care

Palliative care is specialized medical care that focuses on the relief of pain and other symptoms of a serious disease. 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 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 of people with cancer and their families. Palliative care is not the same as hospice care or end-of-life.

Coping and support

Learning is a life-threatening illness can be devastating. You will not find easy answers to 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 can be more confident in making treatment choices. Ask about trusted sources of more information.
  • Keep friends and family close. Keep your close relationships strong will help you deal with your cancer. Friends and family can provide the practical support you need, such as help to take care of your house if you're in the hospital. And that you can serve as emotional support when you feel overwhelmed.
  • Find someone to talk to. Although friends and family can be your best allies, in some cases, they have difficulties to deal with the impact of his diagnosis. In these cases, talk with a counselor, medical social worker, or a pastoral or religious counselor may 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.Have a life-threatening illness, such as cancer, requires you to prepare for the possibility that you could 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 the end-of-life, if necessary.

Make plans for the unknown. Have a life-threatening illness, such as cancer, requires you to prepare for the possibility that you could 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 the end-of-life, if necessary.

Preparing for your appointment

Start by making an appointment with your doctor if you have any of the signs or symptoms that worry you. If the 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 a doctor who specializes in the treatment of cancer (oncologist).

What you can do

  • Be aware of any pre-appointment restrictions, such as the restriction of the diet.
  • List of symptoms, including any that seem unrelated to the reason for which you scheduled the appointment.
  • The list of personal information, including any recent changes or stressors.
  • List of all the medications, vitamins and supplements, including doses.
  • Ask a relative or friend to accompany you, to help you remember what the doctor says.
  • List of 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 performed at another facility, we ask that the files containing the images are placed on a CD and bring it with you to your appointment.

Some basic questions to ask your doctor include:

  • Do I have bile duct cancer? What kind?
  • What is the stage of my cancer?
  • What does my pathology report say? I have a copy of the pathology report?
  • More evidence is needed?
  • What are my treatment options?
  • What are the possible side effects of each treatment option?
  • There is a treatment you think is best for me?
  • How will my treatment affect my daily life?
  • How long can I take to make my decision about bile duct cancer treatment?
  • What is your experience with the bile duct cancer diagnosis and treatment? How many of the surgical procedures for this type of cancer is performed every year in 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 sites do you recommend?

In addition to the questions that you've prepared to ask your doctor, do not hesitate to ask questions during your appointment.

What to expect from your doctor

Your doctor may ask you a series of questions, such as:

  • When did you first begin experiencing symptoms?
  • How severe are the symptoms? Are occasional or continuous?
  • Is there something to improve or worsen your symptoms?
Symptoms and treatment of Cholangiocarcinoma (bile duct cancer)