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.

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.

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

• Yellowing of your skin and the whites of your eyes (jaundice)
• Intensely itchy skin
• White-colored stools
• Fatigue
• Abdominal pain
• Unintended weight loss

Causes

Cholangiocarcinoma occurs when cells in the bile ducts develop changes (mutations) in their DNA-the material that provides instructions for every chemical process in your body. DNA mutations cause changes in the instructions. One result is that cells may begin to grow out of control and eventually form a tumor — a mass of cancerous cells. It's not clear what causes the genetic mutations that lead to cancer.

Risk factors

Factors that may increase your risk of cholangiocarcinoma include:

• Primary sclerosing cholangitis
• Chronic liver disease
• Bile duct problems present at birth
• A liver parasite.
• Older age
• Smoking

Prevention

There's no way to prevent cholangiocarcinoma (bile duct cancer). But you may reduce your risk of the disease if you:

• Stop smoking.
• Reduce your risk of liver disease

For instance, to reduce your risk of liver inflammation (cirrhosis), drink alcohol in moderation, if at all. Maintain a healthy weight. When working with chemicals, follow the safety instructions on the container.

Diagnosis

• Liver function tests
• Tumor marker test
• A test to examine your bile duct with a small camera
• Imaging tests
• A procedure to remove a sample of tissue for testing

Treatment

Treatments for cholangiocarcinoma (bile duct cancer) may include:

• Surgery
• Liver transplant
• Chemotherapy
• Radiation therapy
• Photodynamic therapy
• Biliary drainage

Because cholangiocarcinoma is a very difficult type of tumor to treat, don't hesitate to ask about your doctor's experience with treating the condition. If you have any doubts, get a second opinion.

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