Things to know about bile duct cancer (cholangiocarcinoma)
Jaundice, abdominal pain, and bloating are symptoms and signs of bile duct cancers.
Bile duct cancer arises from the cells that line the bile ducts, the drainage system for bile that is produced by the liver. Bile ducts collect this bile, draining it into the gallbladder and finally into the small intestine where it aids in the digestion process. Bile duct cancer is also called cholangiocarcinoma.
Bile duct cancer is a rare form of cancer, with approximately 2,500 new cases diagnosed in the United States each year. There are three general locations where this type of cancer may arise within the bile drainage system:
- Within the liver (intrahepatic) affecting the bile ducts located within the liver
- Just outside of the liver (extrahepatic or perihilar) located at the notch of the liver where the bile ducts exit
- Far outside of the liver (distal extrahepatic) near where the bile ducts enter the intestine (called the ampulla of Vater)
Bile duct cancers are most commonly found just outside of the liver in the perihilar area and least commonly found within the liver.
Sign of Bile Duct Cancer
Jaundice
Jaundice, also referred to as icterus, is the yellow staining of the skin and sclerae (the whites of the eyes) by abnormally high blood levels of the bile pigment, bilirubin. The yellowing extends to other tissues and body fluids and also may turn the urine dark. Yellowing of only the skin also can be caused by eating too many carrots or drinking too much carrot juice.
Jaundice can occur whenever this normal process of destruction of red blood cells and elimination of bilirubin is interrupted. This occurs when there is abnormally increased destruction of red blood cells (hemolysis), liver disease that reduces the ability of the liver to remove and modify bilirubin, or obstruction to the flow of bile into the intestine.
Read more about causes of jaundice »
What are causes and risk factors for bile duct cancer?
Depending upon where the bile duct blockage occurs, this can lead to inflammation of the liver (hepatitis) and/or pancreas (pancreatitis).
The incidence of bile duct cancer increases with age. It is slow-growing cancer that invades local structures and for that reason, the diagnosis is often made late in the disease process when the bile ducts become blocked. This blockade prevents bile drainage from the liver into the gallbladder and intestine. Depending upon where the blockage occurs, this can lead to inflammation of the liver (hepatitis) and/or pancreas (pancreatitis).
Most patients who develop bile duct cancer have no risk to do so. However, chronic inflammation of the bile ducts may be a risk factor for this cancer. Diseases that can cause this type of chronic inflammation include primary sclerosing cholangitis (especially when associated with ulcerative colitis), chronic liver disease, including hepatitis B, hepatitis C, chronic alcoholic hepatitis, and cirrhosis.
Certain parasitic infections found in the Far East that cause liver infections are associated with an increased risk.
Gallstones are not a risk factor for developing bile duct cancer, but stones within the liver do pose an increased risk. Liver stones are not often seen in the North American population but are more common in Asian countries.
There are rare congenital diseases that increase the risk of bile duct cancer, including Lynch II syndrome (hereditary nonpolyposis colorectal cancer associated with biliary tree and other cancers) and Caroli's syndrome (portal hypertension, hepatic fibrosis, and biliary tree cysts).
Native Americans are six times more likely to develop bile duct cancer. Asian Americans may also be at higher risk. Bile duct cancer is also more prevalent in Israel and Japan, but it is a very rare disease in North America.
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Cancer is the result of the uncontrolled growth of abnormal cells anywhere in the body.
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What are bile duct cancer symptoms and signs?
The initial symptoms of bile duct cancer are jaundice, itching, abdominal pain, bloating, and weight loss.
The initial symptoms of bile duct cancer occur because of the inability of bile to drain normally from the liver where it is produced. This causes liver inflammation (hepatitis). Cholangiocarcinoma symptoms include yellow coloring of the skin and eyes (jaundice), itching, abdominal pain, bloating, and weight loss. Low-grade fever may be present, and there can be darkening in the color of urine and stool.
Unfortunately, bile duct tumors may not cause any symptoms until they have grown in size and cancer has spread (metastasized) from beyond its original location. Abdominal pain is often a late symptom and is usually located in the right upper quadrant of the belly and may be associated with a tender, enlarged liver.
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What is the prognosis for bile duct cancer? What is the life expectancy for bile duct cancer?
How well a patient does after the diagnosis of bile duct cancer depends upon many factors, including where the tumor is located, if and how much it has spread, and the patient's underlying general health. Patients have a better prognosis the farther away from the liver hilum the tumor is located, and according to certain aspects of shape and cell type within the tumor. Prognosis is worse for those patients whose tumor has invaded adjacent tissues, has lymph node involvement, or has spread to distant places in the body.
If untreated, bile duct cancer survival is 50% at one year, 20% at two years, and 10% at three years with virtually no survival at five years.
Being able to completely remove the tumor increases survival but this mostly depends upon the location of the tumor and whether it has invaded other tissues.
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Is it possible to prevent bile duct cancer?
Since the cause of bile duct cancer is uncertain, specific methods of prevention do not exist. However, preventing liver inflammation and cirrhosis may decrease the risk of developing this cancer. This includes moderating the use of alcohol, being vaccinated for the hepatitis B virus, and abstaining from risky behaviors that might cause infection with hepatitis C.
As with all diseases that tend to develop at an older age, living a healthy lifestyle may extend lifespan, as well. This includes not smoking, eating a balanced diet, keeping physically active, and maintaining a healthy weight.
What are the statistics for bile duct cancer?
There are approximately 2,500 new cases of bile duct cancer diagnosed each year in the United States or one case per 100,000 people.
In patients who have bile duct cancer located in the liver hilum, 40%-60% of patients undergo surgery that completely removes the tumor and the average survival is 24 months. For patients with tumor in the same location, but cannot be completely removed, average survival is 21 months.
