Cholangiocarcinoma, which is also known as bile duct cancer, is a cancer that forms in the slender tubes or bile ducts that carry the fluid bile, which helps the body digest fat. Bile ducts connect the liver to the gallbladder as well as to the small intestine. This condition, which is generally uncommon, occurs mostly in people who are over the age of 50. However, it can even appear in younger people.

Unfortunately, cholangiocarcinoma is one of the types of tumor that is difficult to treat.


Cholangiocarcinoma is of three main types:

  • Intrahepatic Cholangiocarcinoma- This type occurs in the parts of the bile ducts that are within the liver and may also be classified as a type of liver cancer.


  • Hilar Cholangiocarcinoma- This occurs in the bile ducts just outside of one’s liver. This type is also known as perihilar cholangiocarcinoma.


  • Distal Cholangiocarcinoma- This type can occur in the portion of the bile duct which is nearest to the small intestine.


Signs and symptoms of this condition can include the following:

  • Yellowing of your skin as well as the white portion of your eyes, i.e. jaundice
  • White-colored stool
  • Fatigue
  • Abdominal pain
  • Intensely itchy skin
  • Unintended weight loss


See your doctor if you continue to have persistent fatigue, abdominal pain, jaundice, or any such signs and symptoms that you are finding bothersome. He/she may refer you to a specialist in digestive diseases i.e. a gastroenterologist.

Causes & risk factors

Cholangiocarcinoma generally occurs when the cells in the bile ducts develop changes in their DNA. Your DNA is the material that provides instructions for every chemical process in the body. DNA mutations can change the instruction. One result is that cells might begin to grow out of control, and eventually form a tumor i.e. a mass of cancerous cells.

It is not yet clear what exactly leads to the genetic mutations leading to cancer.

Some of the factors that can increase the risk of cholangiocarcinoma include:

  • Chronic liver disease- Scarring of the liver which can be caused by a history of the chronic liver disease also increases the risk of cholangiocarcinoma.


  • Primary sclerosing cholangitis- This disease can cause hardening and scarring of the bile ducts.


  • Bile duct problems present at birth- People who are born with a choledochal cyst, which can lead to dilated and irregular bile ducts, have an increased risk of having cholangiocarcinoma.


  • A liver parasite- In certain areas of Southeast Asia, cholangiocarcinoma is associated with liver fluke infection. This can occur if one eats raw or undercooked fish.


  • Smoking- Smoking is also associated with an increased risk of cholangiocarcinoma.


  • Older age- Cholangiocarcinoma generally occurs among adults who are over the age of 50.


Cholangiocarcinoma is not inherited but according to studies blood relatives of a person with cholangiocarcinoma might have an increased risk of developing cancer compared to the general population. However, most of the people suffering from this condition do not have any family history of the disease.


If your doctor suspects these conditions, he/she will likely need you to undergo one or more of the following tests:

Liver function tests

Blood tests for measuring your liver function can give your doctor certain clues about what exactly is causing your signs and symptoms.

Tumor marker test

Checking the level of cancer antigen in your blood may also provide your doctor with certain clues about your diagnosis. A high level of antigen in your blood doesn’t necessarily mean you have bile duct cancer. This result can also indicate other bile duct diseases, such as bile duct obstruction or inflammation.

Endoscopic retrograde cholangiopancreatography

During this method, a thin tube which is equipped with a small camera is passed down the throat and through your digestive tract to your small intestine. The camera helps to examine the area where the bile ducts connect to the small intestine. Your doctor can also use this procedure to inject dye into the bile ducts as it can help them show up better on imaging tests.

Imaging tests

Imaging tests let your doctor examine your internal organs to check for abnormalities that may indicate cholangiocarcinoma. Techniques include computerized tomography (CT) scans, magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP). MRCP is nowadays used more as a noninvasive alternative to ERCP. It can offer 3-D images without the need for a dye for enhancing them.


Biopsy is a procedure which involves removing a small sample of tissue for examination under a microscope.

If any suspicious area is located near where your bile duct joins the small intestine, your doctor might obtain a biopsy sample during ERCP. If the suspicious area is located within or near the liver, your doctor will need to obtain the tissue sample by inserting a long needle through your skin to the affected area. This is also known as fine-needle aspiration. He/she might use an imaging test as well, such as endoscopic ultrasound or CT scan, as it can help guide the needle to the area precisely.

How your doctor chooses to perform the biopsy will very likely influence which treatment options are going to be available to you later. For example, if your doctor uses y fine-needle aspiration for performing the biopsy, you will not be legible for liver transplantation.

Treating cholangiocarcinoma is difficult and therefore you should not hesitate to ask about your doctor’s experience with diagnosing this condition. If you have any doubts, you can get a second opinion.

Once your doctor confirms a diagnosis of cholangiocarcinoma, he/she is going to try to determine the extent or the stage of the cancer. This generally involves additional imaging tests. Knowing the stage of the cancer is also going to help in choosing the treatment option.


Treatments for cholangiocarcinoma or bile duct cancer can include any of the following:


Using this method, doctors try to remove as much of the cancer as it is possible for them. For very small bile duct cancers, doctors will need to remove part of the bile duct and join the cut ends. For advanced bile duct cancers, nearby liver tissue, pancreas tissue or lymph nodes might require removal as well.


Chemotherapy involves using drugs to kill cancer cells. Chemotherapy may sometimes also be used before a liver transplant. Sometimes it is also an option for people with advanced cholangiocarcinoma as it can help in slowing the disease and relieving any signs and symptoms.

Radiation therapy

Radiation therapy makes the use of high-energy sources, like photons and protons, to damage and destroy the cancer cells. Radiation therapy generally involves a machine that directs radiation beams at your body. This is known as external beam radiation. Or it can also involve placing radioactive material inside the body near the site of your cancer. This is known as brachytherapy.

Liver transplant

Surgery that involves removing a liver and replacing it with one from a donor is known as a liver transplant. This is an option in certain cases for people with hilar cholangiocarcinoma. For many, a liver transplant is generally a cure for hilar cholangiocarcinoma. However, there is a risk that the cancer might recur after the transplant.

Photodynamic therapy

In this method, a light-sensitive chemical is injected into a vein and it accumulates in the fast-growing cancer cells. Laser light which is directed at the cancer causes a chemical reaction in the cancer cells and thus causes them to die. Typically, multiple treatments are required. Photodynamic therapy can help in relieving your symptoms, and it may also slow the growth of cancer. After the treatments, you will need to avoid exposure to the sun.

Cholangiocarcinoma is a difficult type of tumor to treat, and therefore you must not hesitate to ask about your doctor’s experience in treating the condition. If you have any doubts, you should get a second opinion.


There’s no way in which you can entirely prevent cholangiocarcinoma.

However, taking certain steps can reduce the risk of the disease.

Stop smoking- Smoking is linked to an increased risk of this condition. If you smoke, it is best to stop. If you’ve tried to quit in the past and haven’t been successful, you can discuss this with your doctor.

Reduce your risk of liver disease- Chronic liver disease is also associated with an increased risk of cholangiocarcinoma. Some causes of liver disease are not preventable. But you should take proper care of your liver.

For example, to reduce your risk of liver inflammation, avoid alcohol or drink in moderation. It is best to maintain a healthy weight. When working with chemicals, it is also important to follow the safety instructions on the container.


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