Bile duct cancer

An overview

Cholangiocarcinoma is cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile. Bile ducts connect your liver to your gallbladder and to your small intestine. This condition, also known as bile duct cancer, is an uncommon form of cancer that occurs mostly in people older than age 50, though it can occur at any age. The small ducts come together to form the right and left hepatic ducts, which lead out of the liver. The two ducts join outside the liver and form the common hepatic duct. The cystic duct connects the gallbladder to the common hepatic duct. Bile from the liver passes through the hepatic ducts, common hepatic duct, and cystic duct and is stored in the gallbladder.
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

Types of bile duct cancer

Doctors divide cholangiocarcinoma into different types based on where the cancer occurs in the bile ducts:
• Intrahepatic cholangiocarcinoma occurs in the parts of the bile ducts within the liver and is sometimes classified as a type of liver cancer. Only a small number of bile duct cancers are intrahepatic. Intrahepatic bile duct cancers are also called intrahepatic cholangiocarcinomas.
• Extrahepatic bile duct cancer: This type of cancer forms in the bile ducts outside the liver. The extrahepatic bile duct is made up of the hilum region and the distal region. Cancer can form in either region.
• Hilar cholangiocarcinoma occurs in the bile ducts just outside of the liver. This type is also called perihilar cholangiocarcinoma.
• Distal cholangiocarcinoma occurs in the portion of the bile duct nearest the small intestine.

Symptoms for bile duct cancer

Signs and symptoms of cholangiocarcinoma include:
• Yellowing of your skin and the whites of your eyes (jaundice)
• Intensely itchy skin
• Dark urine
• White-colored stools
• Fatigue
• Clay coloured stool
• Abdominal pain
• Unintended weight loss
• Fever

Causes of bile duct cancer

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
Bile duct stones. These are similar to gallstones but much smaller.
Choledochal cysts. Some people are born with a rare condition that causes bile-filled sacs along your bile ducts. Without treatment, they may lead to bile duct cancer.
Liver fluke infection. This is rare in the U.S. but more common in Asia. It happens when people eat raw or poorly cooked fish that’s infected with tiny parasitic worms called liver flukes. They can live in your bile ducts and cause cancer.
Reflux. When digestive juices from your pancreas flow back into your bile ducts, they can’t empty properly.
Cirrhosis. Alcohol and hepatitis can damage your liver and cause scar tissue, raising the risk of bile duct cancer.
• Inflammatory bowel disease (including Crohn’s disease and ulcerative colitis)
• Obesity
• Diabetes
• Viral hepatitis
• Drinking a lot of alcohol


Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
Liver function tests: A procedure in which a blood sample is checked to measure the amounts of bilirubin and alkaline phosphatase released into the blood by the liver. A higher than normal amount of these substances can be a sign of liver disease that may be caused by bile duct cancer.
Laboratory tests: Medical procedures that test samples of tissue, blood, urine, or other substances in the body. These tests help to diagnose disease, plan and check treatment, or monitor the disease over time.
Carcinoembryonic antigen (CEA) and CA 19-9 tumor marker test: A procedure in which a sample of blood, urine, or tissue is checked to measure the amounts of certain substances made by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the body. These are called tumor markers. Higher than normal levels of carcinoembryonic antigen (CEA) and CA 19-9 may mean there is bile duct cancer.
Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs, such as the abdomen, and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
Laparoscopy: A surgical procedure to look at the organs inside the abdomen, such as the bile ducts and liver, to check for signs of cancer. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as taking tissue samples to be checked for signs of cancer.
Percutaneous transhepatic cholangiography (PTC): A procedure used to x-ray the liver and bile ducts. A thin needle is inserted through the skin below the ribs and into the liver. Dye is injected into the liver or bile ducts and an x-ray is taken. A sample of tissue is removed and checked for signs of cancer. If the bile duct is blocked, a thin, flexible tube called a stent may be left in the liver to drain bile into the small intestine or a collection bag outside the body. This procedure may be used when a patient cannot have surgery.


The prognosis and treatment options depend on the following:
• Whether the cancer is in the upper or lower part of the bile duct system.
• The stage of the cancer (whether it affects only the bile ducts or has spread to the liver,lymph nodes, or other places in the body).
• Whether the cancer has spread to nearby nerves or veins.
• Whether the cancer can be completely removed by surgery.
• Whether the patient has other conditions, such as primary sclerosing cholangitis.
• Whether the level of CA 19-9 is higher than normal.


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