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What is Bile Duct Cancer?
Bile duct cancer begins when normal cells in the bile duct change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).
About the bile duct
The bile duct is a 4-inch to 5-inch long tube that connects the liver and gallbladder to the small intestine. The bile duct allows bile, which is made in the liver and stored in the gallbladder, to flow into the small intestine. Bile is a liquid that helps to break down fats found in foods and helps the body get rid of the waste material that is filtered out of the bloodstream by the liver.
The bile duct starts in the liver. Within the liver, smaller tubes (similar to small blood vessels) drain bile from the cells in the liver into larger and larger branches, ending in a tube called the common bile duct. Outside of the liver, the bile duct drains into the small intestine. The gallbladder is a reservoir that holds bile until food reaches the intestines. It is attached by a small duct, called the cystic duct, to the common bile duct about one-third of the way down the bile duct from the liver. The end of the bile duct empties into the small intestine.
Types of bile duct cancer
Cancer can occur in any part of the bile duct. For bile duct cancer, doctors look at the exact location of the tumor:
Extrahepatic. The part of the bile duct that is outside of the liver is called extrahepatic. It is in this part of the bile duct where cancer usually begins. A common site for bile duct cancer is at the point where the right and left hepatic ducts join. A tumor that starts in this area is also sometimes called a Klatskin's tumor. The rest of the bile duct cancers that begin outside the liver occur between where the right and left hepatic ducts meet and where the bile duct empties into the small intestine.
Intrahepatic. About 5% to 10% of bile duct cancers are intrahepatic, or inside the liver.
Symptoms and Signs
People with bile duct cancer may experience the following symptoms or signs, usually because the tumor is blocking the bile duct. Sometimes, people with bile duct cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.
One common symptom is jaundice, which is a yellowing of the skin and the whites of the eyes. When the bile duct is blocked, the liver cannot excrete bile, and the bile backs up into the bloodstream. (The blockage may not be cancer; it can also be caused by a gallstone or scar tissue.) Bile contains bilirubin, which is dark yellow and can cause the skin and whites of the eyes to turn yellow if there are higher levels of it in the bloodstream. A person's urine may also become a dark color, and bowel movements may become pale.
However, it is important to note that jaundice is a common symptom for many conditions so there can be many causes. Your doctor may need to do several diagnostic tests to find the exact cause. Many diseases associated with jaundice are not serious or life threatening; bile duct cancer is one of the less common causes.
In addition to jaundice, other symptoms of bile duct cancer include:
Itching, caused by a buildup of bile salts and bilirubin in the body and deposited in the skin
Loss of appetite
Abdominal pain. Early bile duct cancer usually does not cause pain, but a person may experience pain if the cancer has spread.
Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you've been experiencing the symptom(s) and how often.
If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.
Risk Factors and Prevention
A risk factor is anything that increases a person's chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.
The following factors may raise a person's risk of developing bile duct cancer:
Previous disease or irritation of the bile duct. Inflammation of the bile duct can be caused by ulcerative colitis or stones similar to gallstones. Diseases and conditions that increase the risk of bile duct cancer include:
Primary Sclerosing Cholangitis (PSC), which is a rare inflammatory condition of the bile ducts with no known cause.
Choledochal cyst, which is an abnormality a person is born with that causes a swollen area on the part of the bile duct outside the liver.
Caroli's syndrome, which is an abnormality of the small bile ducts within the liver that a person is born with that increases the risk of infection and stones, like gallstones, forming in the liver.
Cirrhosis, which is liver disease that can cause scarring or long-lasting irritation.
Infection with liver flukes that invade the bile duct.
Age. Older adults are more likely to develop bile duct cancer.
Certain chemicals. Dioxins, nitrosamines, and polychlorinated biphenyls (PCBs) may cause bile duct cancer. In particular, people who work in rubber plants and automotive industries may be exposed to these chemicals.
Reducing Your Risk
Even though some people who have no risk factors develop bile duct cancer, there are ways to lower your individual risk of bile duct cancer.
Avoid coming in contact with hazardous chemicals. Even though thorium dioxide (a chemical, previously used in x-ray examinations, associated with a high risk of developing bile duct cancer) has been banned from use, other hazardous chemicals are still available or found in the environment.
Avoid alcohol abuse, which can lead to cirrhosis.
If traveling in parts of the world where liver flukes are common, drink only purified water and foods that have been thoroughly cooked.
Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
Type of cancer suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose bile duct cancer or distinguish it from other possible causes of jaundice:
Blood chemistry tests. Blood chemistry tests measure the levels of bilirubin and alkaline phosphatase and test other liver functions. High levels of these substances could indicate that the bile duct is not working well.
Tumor marker tests (CEA and CA19-9). Tumor markers are substances found in higher than normal amounts in the blood, urine, or tissues of people with certain types of cancer. Bile duct cancer may cause high levels of carcinoembryonic antigen (CEA) and CA19-9 in the blood. However, a person can have bile duct cancer even if there are normal levels of these markers. Also, high levels of these substances can sometimes occur from diseases other than cancer.
Other tests may be performed to provide more information about the bile duct. These include:
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
The type of biopsy performed depends on the location of the tumor. The doctor can obtain tissue samples during a procedure called a percutaneous transhepatic cholangiography (PTC) or another procedure called an endoscopic retrograde cholangiopancreatography (ERCP). PTC and ERCP are described below. Or, a sample will be taken by using a computed tomography scan (CT or CAT scan; see below) to guide a thin needle biopsy through the skin into the possible tumor to collect a sample of cells.
Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. Occasionally, a biopsy is not possible. In this uncommon situation, people will be treated for bile duct cancer based on other test results and symptoms.
Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. During an ultrasound, the doctor may be able to see the actual tumor; more often when one of the larger bile ducts is blocked, the small bile ducts behind the blockage get larger. It is this “dilation of ducts” that can be seen on an ultrasound.
To view the bile duct, the doctor may use an endoscopic (a thin, flexible tube inserted through the mouth) or laparoscopic (see below) ultrasound. Both of these procedures allow for a clearer view of the bile duct and can help the doctor perform a biopsy.
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail. CT scans can also be used to guide a needle biopsy.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. An MRI specific for the bile duct is called MRI cholangiopancreatography. A contrast medium may be injected into a patient's vein to create a clearer picture.
PTC. In this test, a thin needle is inserted into the bile duct in the liver. The doctor injects contrast medium into the needle, which flows into the bile duct. An x-ray is used to show whether the bile duct is blocked and, if so, locate the site of the blockage. This is important in planning treatment.
ERCP. The doctor inserts a flexible tube down the person's throat, through the stomach, and into the bile duct. Dye is injected into the tube, which will help outline the bile duct on an x-ray. A tiny brush can also be inserted through the tube to collect cells and tissue fragments for a biopsy. This technique can help to find and take a sample of the tumor and provide information that is important in planning treatment.
Laparoscopy. The doctor views the bile duct, gallbladder, and liver through a lighted tube inserted into a surgical opening in the person's abdomen. Some laparoscopes can help the doctor to work with small instruments through the tube to take a tissue sample.
Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.
The method used to stage other types of cancers, called the TNM classification (Stage I through IV), is medically complex for bile duct cancer. A simpler way to describe the stages of bile duct cancer is under the headings listed below. By classifying each cancer into one of these categories, the health care team can then plan the best treatment strategy.
Local. This type of bile duct cancer is located only within the bile duct and can be removed surgically. There is no evidence of any spread to areas outside of the bile duct.
Locally advanced. This type is still located only in the area around the bile duct, but does affect nearby organs, arteries, and/or veins. There is no evidence of spread to any distant parts of the body.
Metastatic. The tumor has spread beyond the area of the bile duct to reach distant parts of the body. It is unlikely that surgery can remove the cancer.
Recurrent. Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.
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