Bile Duct Cancer (Cholangiocarcinoma) – Overview | Gastroenterology

Bile Duct Cancer (Cholangiocarcinoma)

What is bile duct cancer?

Bile duct cancer, also known as cholangiocarcinoma, occurs when abnormal cells grow out of control within the bile ducts. These are thin tubes about 4 to 5 inches long that carry a fluid called bile from the liver to the gallbladder and small intestine. Bile encourages you to digest fats in the food you eat.

For some people, treatment can eliminate cancer. In other cases, it may not go away completely. You may require ordinary dosages of chemotherapy, radiation, or different therapies to hold it under control.

Knowing more about the condition and getting support from your medical team and loved ones can help you manage stress and worries about the future.

Bile duct cancer stages

The TNM system, established by the American Joint Committee on Cancer, is most commonly used in the stage of bile duct cancer. The TNM system uses three main factors in staging cancer:

T (tumor): This depicts the size of the first tumor.

N (node): This indicates whether the cancer is located in the lymph nodes.

M (metastasis): It indicates whether cancer has spread to other parts of the body.

Each worker is assigned a number (0-4) or letter X. A higher number indicates an increased risk. For example, a T1 score indicates a tumor smaller than a T2 grade. The letter X means that the information cannot be evaluated.

Once the T, N, and M grades are assigned, the cancer is staged accordingly:

Stage 0: Cancer is only found in the innermost layer of the bile duct (mucosa) and has not spread to the deeper layers. At this stage, the disease has not invaded the lymph nodes or any other distant sites in the body.

Stage I (stage 1 bile duct cancer): The cancer is still trapped within the bile duct and now extends into the deeper layers of the bile duct wall (the layer of muscle or fibrous tissue). It has not spread to the lymph hubs or other far off destinations.

Stage II (stage II bile duct cancer): Cancer has now spread through the bile duct wall into nearby fat (IIB) or liver tissue (IIB).

Stage III (stage 3 bile duct cancer): Stage III bile duct cancer is isolated into two subcategories:

  • Stage IIIA: The disease has spread to the main portal vein, the common hepatic artery, duodenum (the first part of the small intestine), colon, stomach, or abdominal wall, but not beyond that. Cancer has not spread to the lymph hubs or removed destinations.
  • Stage IIIB: Cancer cells are found in nearby lymph nodes, but cancer has not spread to distant sites.

Stage IV (stage 4 bile duct cancer): Stage 4 cholangiocarcinoma is divided into two subcategories:

  • Stage IVA: Cancer grows and has spread to major blood vessels and lymph nodes but not to distant sites.
  • Stage IVB: Cancer has spread to the lymph nodes away from the tumor or has spread to distant sites.

Bile duct cancer types

Doctors identify bile duct cancer based on where it appears:

  • Outside your liver (extrahepatic): This typically occurs more often and is more treatable. Cancer may form in one of two areas.
    • The hilum area, where your left and right bile ducts meet together to form the common hepatic duct. This is called percutaneous cancer.
    • The distal region, where the common bile duct passes through the pancreas. This is called distal carcinoma.
  • Inside your liver (intrahepatic): This type makes up 5% to 10% of cases.

What are the symptoms of cholangiocarcinoma?

Your symptoms may vary depending on the location of your tumor, but they may include the following:

  • Jaundice, which is a yellowing of the skin, is the most common symptom. This may happen at an early or late stage, depending on the location of the tumor.
  • Dark urine and pale stools may develop.
  • Itching may occur, and it may be caused by jaundice or cancer.
  • You could have pain in your abdomen that cuts through your back. This tends to happen as cancer progresses.

Additional rare but serious side effects may include an enlarged liver, spleen, or gallbladder.

You may also have more general symptoms, such as:

  • Chills
  • Weight loss
  • Fatigue
  • Fever
  • Loss of appetite

Causes of bile duct cancer

Cholangiocarcinoma occurs when cells in the bile ducts cause changes (mutations) in their DNA – the substance that provides instructions for every chemical process in your body. DNA mutations cause changes in the instructions. One of the findings is that the cells may begin to grow out of control and eventually form a tumor – a mass of cancer cells. It is not clear what causes the gene mutations that lead to cancer.

Treating bile duct cancer

Your doctor will advise you about the best cancer treatment. This will depend on the type of cancer you have, whether and to what extent it has spread, your general health, and your treatment preferences.

It may include your treatment options:

  • Surgery
  • Chemotherapy
  • Radiotherapy
  • Photodynamic therapy.

Surgery for bile duct cancer

It is the main treatment for bile duct cancer. Surgery to remove bile duct cancer can be complicated and not suitable for everyone. If surgery is an option for you, it is important that you get a referral to a surgeon interested in these rare types of cancers. The type of operation you will undergo depends on the size of cancer and whether it has spread to nearby tissues.

May include operations:

  • Removal of the bile ducts: This is an option for the early stages of cancer
  • Liver resection: If cancer has spread to the liver, the bile ducts and the affected part of the liver can be removed.
  • The Whipple procedure: This procedure is done if cancer has spread to nearby tissues and organs. It is major surgery and you will need to stay in the hospital for at least two weeks. It removes the bile ducts, part of the stomach, small intestine, pancreas, gallbladder, and adjacent lymph nodes
  • Surgery to remove the blockage: This does not cure or cure cancer but rather helps relieve symptoms caused by the blockage. A tube (stent) may be inserted to help the bile drain.

Chemotherapy for bile duct cancer

You may receive chemotherapy to help treat cancer. You may get it alone or with radiation therapy. Chemotherapy is the use of anti-cancer drugs. It may be used to increase the chance of a cure or to shrink cancer when a cure is not possible. When cancer cannot be cured, chemotherapy can improve survival, reduce symptoms, and improve quality of life.

The goal is to destroy all cancer cells while doing minimal damage to normal cells. The drugs work by stopping the cancer cells from growing and multiplying. Chemotherapy is usually given by injection into a vein (intravenous therapy). There are other methods of chemotherapy, including tablets.

Your doctor will discuss treatment options for the type and stage of your cancer. For more information, see Understanding Chemotherapy or call the Cancer Council at 13 11 20 to speak to a nurse who specializes in cancer treatment.

Radiotherapy for bile duct cancer

Radiation therapy uses radiation to destroy cancer cells in a part of the body. Radiation can target cancer sites in your body. Treatment is carefully planned to cause the least possible damage to the normal body tissues around the tumor.

You may undergo internal radiation therapy (brachytherapy) or external radiation therapy, which is radiation therapy that is carried outside the body.

Radiation therapy cannot cure bile duct cancer but it can be given to help:

  • Relieving symptoms caused by cancer
  • Shrinking the size of cancer to make it easier to remove with surgery
  • Shrinking cancer and controlling its growth.

Photodynamic therapy for bile duct cancer

This drug is used to make cells sensitive to light. The medicine is injected into a vein. It spreads throughout the body and is primarily attracted to cancer cells. It does not become effective until exposed to laser light. It is used in cancer of the bile duct to help relieve symptoms.

The medication is usually injected two days before the ERCP procedure. During this procedure, an endoscope, which contains a small tube with red laser light at its end, is placed in the bile duct. This laser light activates the drug and kills the cancer cells.

After the dye is injected, you may be sensitive to light for a few days. Your doctor will discuss this with you and tell you what to do, such as stay out of the sun. You may also feel some pain, inflammation, or bleeding from the bile duct after this procedure.

Risk factors for bile duct cancer

Elements that may expand your danger of cholangiocarcinoma include:

  • Primary sclerosing cholangitis: This illness causes solidifying and scarring of the bile ducts.
  • Chronic liver disease: Scarring of the liver caused by a chronic liver history increases the risk of developing cholangiocarcinoma
  • Problems of the bile duct are present at birth: People born with a bile cyst, which causes dilated and irregular bile ducts, have a higher risk of developing cholangiocarcinoma.
  • Liver parasite: In regions of Southeast Asia, cholangiocarcinoma is associated with liver fluke infection, which can occur from eating raw or undercooked fish.
  • Cholangiocarcinoma occurs most often in adults over the age of 50.
  • Smoking is associated with an increased risk of cholangiocarcinoma.

Diagnosis of bile duct cancer

A disease of the bile duct can be hard to analyze, so you may require a few tests, including:

  • Blood tests
  • Ultrasound scans
  • Computerized tomography (CT) scans
  • Magnetic resonance imaging (MRI) scans

For some of these tests, you may need to be injected with a special dye that highlights the bile ducts.

It may also need a biopsy. This involves removing a small sample of tissue so that it can be studied under a microscope. However, in some cases, your surgeon may prefer to remove a suspected tumor based on the results of your tests alone.

Prevention of bile duct cancer

There is no way to prevent cholangiocarcinoma (bile duct cancer). But you can reduce your risk of getting sick if you are:

  • Stop smoking: Smoking is related to an expanded danger of cholangiocarcinoma. If you smoke, stop. If you’ve tried to quit smoking in the past and have not succeeded, talk to your doctor about strategies to help you quit.
  • Reduce your risk of developing liver disease: Chronic liver disease is associated with an increased risk of developing cholangiocarcinoma. Some causes of liver disease cannot be prevented, but others can be prevented. Do what you can to deal with your liver.
  • For example, to reduce your risk of developing hepatitis (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 included data for nearly 4,800 people. More study is needed to confirm that long-term use of aspirin is safe for preventing cancer.

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