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What is Cholangiocarcinoma?

Cholangiocarcinoma (CCA), or bile duct cancer, is a type of rare cancer that grows in the small tubes that carry bile, a fluid that helps with digestion. These tubes are called the bile ducts. The bile ducts connect the liver to the gall bladder and small intestine.  

There are three types of cholangiocarcinoma that are named for where they grow in the bile ducts:

  • Intrahepatic cholangiocarcinoma grows in the parts of the bile ducts that are in the liver. This is the least common form of cholangiocarcinoma and accounts for only 10% of all cases.
  • Hilar cholangiocarcinoma grows in the bile ducts right outside of the liver. It is also called perihilar cholangiocarcinoma.
  • Distal cholangiocarcinoma grows in the parts of the bile ducts near the small intestine.

Sometimes, hilar cholangiocarcinoma and distal cholangiocarcinoma are grouped together and called extrahepatic cholangiocarcinoma. 

How common is cholangiocarcinoma?

Cholangiocarcinoma is rare. On average, it affects fewer than 6 in 100,000 people around the world. In the United States, cholangiocarcinoma affects about 8,000 people per year. In some places, though, cholangiocarcinoma is more common. For example, in northeast Thailand, cholangiocarcinoma occurs in about 110 per 100,000 people. Most people diagnosed with cholangiocarcinoma are above the age of 65. Cholangiocarcinoma is slightly more common in men than women.

How is cholangiocarcinoma diagnosed?

Most people with cholangiocarcinoma do not have symptoms when it first starts. Later, when the tumor gets larger, symptoms can include:

  • Yellow skin and eyes (jaundice)
  • Itching
  • Unintentional weight loss
  • Abdominal pain

Lab Tests: If you have symptoms of cholangiocarcinoma, your doctor will order lab tests of your blood to check your bilirubin levels. Bilirubin is a substance in your blood that may be a sign that something is wrong with your liver, bile ducts, or gallbladder. Your doctor may also look for liver enzymes in your blood, called a liver function test.

Imaging: Your doctor will use imaging scans such as ultrasound, CT, MRI, and X-rays to look at where the tumor is and how big it is. They will also check for signs that the tumor has spread to other parts of the body. Your doctor may also use endoscopy to look inside the body. 

Biopsy: To check if the tumor is cholangiocarcinoma your doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is. A sample of your tumor may also be taken during endoscopy.

How is cholangiocarcinoma treated?

Treatment for each patient will be unique. You should go to an expert in cholangiocarcinoma treatment to decide the best approach for you. You can contact MyPART for help finding experts near you.

Treatment options to discuss with your doctor include: 

Surgery: In some cases, cholangiocarcinoma can be removed with surgery. This is the best chance for cholangiocarcinoma to be cured. Surgery to remove cholangiocarcinoma is complex, so you will want to go to a surgeon with experience in bile duct surgery. 

Radiation therapy: Radiation therapy can be used before or after surgery. Or it can be the main treatment if surgery is not an option. External radiation therapy uses a beam of energy aimed at the tumor. Internal radiation therapy uses small pieces of radioactive material placed inside the tumor to kill the tumor cells.

Chemotherapy: Chemotherapy can be used with radiation therapy. Chemotherapy is often used when cholangiocarcinoma has spread to other parts of the body. 

Targeted therapy: Targeted therapy is aimed at the changes in cancer cells that help them grow, divide, and spread. In some cholangiocarcinomas, the cancer cells have changes in the FGFR2 gene, which helps the cancer cells grow out of control. In these cases, a FGFR2 inhibitor (pemigatinib) can help treat cholangiocarcinoma. 

Immunotherapy: Immunotherapy helps the body’s immune system fight cancer cells. Doctors are studying if immunotherapy can help treat cholangiocarcinoma in clinical trials.

Does cholangiocarcinoma run in families?

Some studies show that blood relatives of some people with cholangiocarcinoma may have a higher chance of developing it compared to others. But most people with cholangiocarcinoma have no family history of it. 

How does cholangiocarcinoma form?

Scientists have found several changes in important genes in cholangiocarcinoma, such as FGFR2 and IDH1, that may help cancer cells grow out of control. 

What is the prognosis for someone with cholangiocarcinoma?

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as: 

  • Where the tumor is in your body
  • If the cancer has spread to other parts of your body
  • How much of the tumor was taken out during surgery 

Doctors estimate cholangiocarcinoma survival rates by how groups of people with this cancer have done in the past. Because there are so few people with cholangiocarcinoma, these rates may not be very accurate. The survival rates also don’t take into account newer treatments. 

For people with extrahepatic cholangiocarcinoma, 10% are alive 5 years after diagnosis. For people with intrahepatic cholangiocarcinoma that has been found at an early stage, 24% are alive 5 years after diagnosis. But if not found early, 8% are alive 5 years after diagnosis 

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis.