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Cancer of the Bile Duct
Alan P. Venook, MD, Sabrina Selim, MD


Bile is manufactured in the liver and helps the body digest fats. The bile ducts course throughout the liver collecting bile, then travel to the gall bladder where bile is stored. When bile is needed, such as after a fatty meal, it is released into the small intestine via another duct which connects the gallbladder to the small intestine.

Bile duct cancers, also called cholangiocarcinomas, may arise in many locations in and around the liver. They are rare, affecting only 16,600 people in the United States per year. The only definitive treatment is the complete surgical removal of the tumor, which is not often possible. If the cancer cannot be entirely removed, the principal goals of therapy become the relief of symptoms caused by the accumulation of bile, and relief from pain.

How It Spreads
Bile duct cancer tends to spread into the adjacent liver, along the bile duct surface and through the lymph system to lymph nodes in the region of the liver. Tumors in the bile duct leading from the gall bladder to the common bile duct (cystic duct) can spread to involve the gall bladder. Ultimately, other lymph nodes as well as other organs within the abdomen may become involved.

What Causes It
The cause is unknown although people with chronic inflammatory processes such as ulcerative colitis or parasitic infections of the bile ducts, or with congenital abnormalities of the bile ducts are at higher risk for developing this cancer. No one cause has been clearly demonstrated.

Common Signs and Symptoms
There are no signs or symptoms unique to bile duct cancer. Even if the findings associated with bile duct cancer are present, other explanations, such as gallstones, are far more likely. Symptoms generally develop slowly and are often subtle. Jaundice (the skin turning yellow) and itching are the most common signs. Jaundice is caused by the accumulation in the skin of a component of bile (bilirubin) that normally empties into the intestines after traveling through the bile ducts.

Bloating, weight loss, decreased appetite, fever, nausea or an enlarging abdominal mass are all signs that may be attributable to bile duct cancer. Pain usually signifies advanced disease.

Staging and Treatments
A TNM staging system is used for bile duct cancer, but when deciding which treatment option to use there are really only two stages - localized and unresectable disease.

The optimal treatment for bile duct cancer is surgery. Unfortunately, by the time symptoms develop, the cancer has usually spread throughout the bile ducts and into the liver, meaning that the tumor cannot be entirely removed.

Chemotherapy and radiation therapy are occasionally useful to relieve symptoms. Although they have not been shown to be effective in curing the cancer, these measures can be taken to maintain the quality of life.

Stage

Signs and Symptoms

Diagnostic Procedures

Treatments

Survival

Early

Tumor is isolated to the bile duct and is generally too small to be detected

Generally none

No screening methods available

Surgery followed by radiation therapy

Unknown

Resectable

Tumor is confined to the bile duct. It is quite rare to find a bile duct cancer at this limited stage because symptoms of bile flow obstruction are rare

Generally none

Blood tests: hemoglobin (anemia); white blood count; abnormal liver function tests; abnormal clotting (PT, PTT) tests

Ultrasound: Enlarged bile duct due to blockage

MRI: May help determine if bile duct cancer can be surgically removed

PTC: An injected dye followed by x-ray may reveal site of blockage

ERCP: Along with PTC, this may help define the site and cause of obstruction and correctly predict the presence of cancer 90% of the time

Chest x-ray: If cancer is confirmed, this should be done to look for tumor spread to the lung

Surgery: Surgery can be done with hopes of a cure. The extent of surgery depends on the location and size of the tumor. If the tumor is isolated to either the right or left bile duct, it may be possible to remove the tumor and its accompanying lobe of the liver. Tumor involving both ducts or their junction create more problems and more difficult operation. The overall health of the patient is important when determining if surgery is possible. Radiation therapy is often recommended following surgery

Chemotherapy: Studies have not shown that chemotherapy prolongs survival. Mitomycin C or 5-FU, however, may cause tumor shrinkage in 20-25% of patients. Even with shrinkage, however, patients may not be better off as the tumor ultimately regrows and treatment has side effects.

Radiation therapy: If the tumor is fairly small, it may be treated with radiation without much damage to the surrounding liver. It may also apply to patients with small or microscopic deposits of tumor following surgery

5 years: less than 25%

Unresectable

Tumor has spread to organs beyond the bile duct (lymph nodes, liver) and cannot be removed

Jaundice, itching, bile infections, pain, appetite, edema, nausea, insomnia

 

There are no standard treatments, so chemotherapy and/or radiation therapy clinical trials should be considered. Palliative measures, such as relieving symptoms of itching or infection by surgical bypass system for bile may be done.

2 years: less than 1%

Supportive Therapies
Problems associated with jaundice can include severe itching and infections in the bile. If the drainage procedures described above are not effective, itching will often be relieved by Benadryl, Atarax or cholestyramine.
Large doses of narcotics may be needed to relieve pain. Such drugs may have excessive side effects, since they are eliminated by the liver, which may not be functioning properly.
Non-steroidal anti-inflammatory drugs may be surprisingly effective even against the pain associated with bile duct cancer.
Frequent small meals may be necessary to get enough nutrition since an abdominal mass may reduce the size of the stomach.
Water pills (diuretics) can reduce fluid in the abdomen or legs. They may cause significant imbalance in kidney function, however, and can create problems if not carefully monitored and adjusted.
Nausea will often be relieved by standard medications, including suppositories.
Loss of appetite may be helped by a drug called Megace.
Sleep disturbances are common, but most sleeping pills are broken down by the liver, so they should be used carefully.

The Most Important Questions to Ask Your Doctor
Should I see another physician to confirm that this tumor can or cannot be removed?
Can the drainage of my bile ducts be done through my intestines so I won't have to have a tube coming out through my skin?
Am I a candidate for an investigational therapy at another medical center?
How sick will radiation and/or chemotherapy make me, and does the potential benefit make it worthwhile?
Is the treatment worthwhile if the tumor is too advanced for surgery?
Can anything be done to improve the quality of my life?

Reference
Everyone's Guide to Cancer Therapy by Malin Dollinger, Ernest H. Rosenbaum, Margaret Tempero and Sean Mulvihill. Andrews McMeel. 4th edition, 2001




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