Extrahepatic bile duct cancer, a rare cancer, is a disease in which malignant cells are found in the tissues of the extrahepatic bile duct. The bile duct is a tube that connects the liver and the gallbladder to the small intestine. The part of the bile duct that is outside the liver is called the extrahepatic bile duct. A fluid called bile, which is made by the liver and breaks down fats during digestion, is stored in the gallbladder. When food is being broken down in the intestines, bile is released from the gallbladder through the bile duct to the first part of the small intestine.

A doctor should be seen if there are any of the following symptoms:

Yellowing of the skin (jaundice)
Pain in the abdomen
Fever
Itching

If there are symptoms, a doctor will perform an examination and order tests to see if there is cancer. A patient may have an ultrasound, a test that uses sound waves to find tumours. A patient may also have a CT (computed tomographic) scan, which is a special type of x-ray that uses a computer to make a picture of the inside of the abdomen. Another special scan called magnetic resonance imaging (MRI), which uses magnetic waves to make a picture of the inside of the abdomen, may be done as well.

A doctor may perform a test called an ERCP (endoscopic retrograde cholangiopancreatography). During this test, a flexible tube is put down the throat, through the stomach, and into the small intestine. The doctor can see through the tube and inject dye into the drainage tube (duct) of the pancreas so that the area can be seen more clearly on an x-ray.

PTC (percutaneous transhepatic cholangiography) is another test that can help find cancer of the extrahepatic bile duct. During this test, a thin needle is put into the liver through the right side of the patient. Dye is injected through the needle into the bile duct in the liver so that blockages can be seen on x-rays.

If tissue that is not normal is found, the doctor may remove a small amount of fluid or tissue from the bile duct and look at it under the microscope to see if there are any cancer cells. This procedure is called a biopsy and is usually done during the PTC or ERCP.

Because it is sometimes hard to tell whether a patient has cancer or another disease, surgery may be needed to see if there is cancer of the bile duct. If this is the case, the doctor will cut into the abdomen and look at the bile duct and the tissues around it for cancer. If there is cancer and if it looks like it has not spread to other tissues, the doctor may remove the cancer or relieve blockages caused by the tumour.

The chance of recovery (prognosis) and choice of treatment depends on the location of the cancer in the bile duct, the stage of the cancer (whether it is only in the bile duct or has spread to other places), and the patient’s general health.

Stages of extrahepatic bile duct cancer

Once extrahepatic bile duct cancer is found (diagnosed), more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. To plan treatment, a doctor needs to know the stage of the cancer. The following stages are used for extrahepatic bile duct cancer:

Localized

The cancer is only in the area where it began and can be removed in an operation.

Unresectable

All of the cancer cannot be removed in an operation. The cancer may have spread to nearby organs and lymph nodes or to other parts of the body. (Lymph nodes are small bean-shaped structures that are found throughout the body. They produce and store infection-fighting cells.)

Recurrent

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the bile duct or in another part of the body.

How extrahepatic bile duct cancer is treated

There are treatments for all patients with extrahepatic bile duct cancer. Two kinds of treatment are used:

• Surgery (taking out the cancer or taking steps to relieve symptoms caused by the cancer)
• Radiation therapy (using high-dose x-rays to kill cancer cells)

Other treatments for extrahepatic bile duct cancer are being studied in clinical trials. These include:

• Chemotherapy (using drugs to kill cancer cells)
• Biological therapy (using the body’s immune system to fight cancer)

Surgery is a common treatment of extrahepatic bile duct cancer. If the cancer is small and is only in the bile duct, a doctor may remove the whole bile duct and make a new duct by connecting the duct openings in the liver to the intestine. The doctor will also remove lymph nodes and look at them under the microscope to see if they contain cancer. If the cancer has spread outside the bile duct, a surgeon may remove the bile duct and the tissues around it.

If the cancer has spread and it cannot be removed, the doctor may do surgery to relieve symptoms. If the cancer is blocking the small intestine and bile builds up in the gallbladder, the doctor may do surgery to go around (bypass) all or part of the small intestine. During this operation, the doctor will cut the gallbladder or bile duct and sew it to the small intestine. This is called biliary bypass. Surgery or x-ray procedures may also be done to put in a tube (catheter) to drain bile that has built up in the area. During these procedures, the doctor may make the catheter drain through a tube to the outside of the body or the catheter may go around the blocked area and drain the bile to the small intestine. In addition, if the cancer is blocking the flow of food from the stomach, the stomach may be sewn directly to the small intestine so the patient can continue to eat normally.

Radiation therapy is the use of high-energy x-rays to kill cancer cells and shrink tumours. Radiation may come from a machine outside the body (external-beam radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes into the area where the cancer cells are found (internal radiation therapy).

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by inserting a needle into a vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells outside the bile duct.

Biological therapy tries to get the body to fight cancer. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body’s natural defences against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy. This treatment is currently only being given in clinical trials.

Localized Extrahepatic Bile Duct Cancer

Treatment may be one of the following:

1. Surgery to remove the cancer.
2. Surgery to remove the cancer followed by external-beam radiation therapy.

Unresectable Extrahepatic Bile Duct Cancer

Treatment may be one of the following:

1. Surgery or other procedures to bypass blockage in the bile duct.
2. Surgery or other procedures to bypass blockage in the bile duct followed by external-beam radiation therapy or internal radiation therapy.
3. Clinical trials of radiation therapy with drugs to make the cancer cells more sensitive to radiation (radiosensitizers).
4. Clinical trials of chemotherapy or biological therapy.

Recurrent Extrahepatic Bile Duct Cancer

Treatment depends on many factors, including where the cancer came back and what treatment the patient received before. Clinical trials are testing new treatments

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