Cholangiocarcinoma is a rare and often fatal cancer that affects the bile ducts.

The bile ducts are a series of tubes that transport digestive juices called bile from your liver (where it’s made) to your gallbladder (where it’s stored). From the gallbladder, ducts carry bile to your gut, where it helps to break down fats in the foods you eat.

In most cases, cholangiocarcinoma arises in those parts of the bile ducts that lie outside the liver. Rarely, the cancer can develop in ducts that are located within the liver.

Types of cholangiocarcinoma

Most often, cholangiocarcinomas are part of the family of tumors known as adenocarcinomas, which originate in glandular tissue.

Less commonly, they’re squamous cell carcinomas, which develop in the squamous cells that line your digestive tract.

Tumors that develop outside of your liver tend to be fairly small. Those in the liver can be small or large.

What are the symptoms of cholangiocarcinoma?

Your symptoms may vary depending on the location of your tumor, but they can include the following:

• Jaundice, which is yellowing of the skin, is the most common symptom. This may develop at an early or late stage, depending on the tumor site.

• Dark urine and pale stools may develop.

• Itching may occur, and it can be caused by jaundice or by the cancer.

• You can have pain in your abdomen that penetrates to your back. This tends to occur as the cancer progresses.

Additional rare but serious side effects may include enlargement of your liver, spleen, or gallbladder.

You may also have more general symptoms for bile ducts cancer (Cholangiocarcinoma), such as:

• chills

• fever

• loss of appetite

• weight loss

• fatigue

What causes cholangiocarcinoma?

Doctors don’t understand why cholangiocarcinoma develops, but it’s thought that chronic inflammation of the bile ducts and chronic parasitic infections can play a part.

Who is at risk for cholangiocarcinoma?

You’re more likely to develop cholangiocarcinoma if you’re male or older than 65 years of age. Certain conditions can increase your risk for this type of cancer, including:

• liver fluke (parasitic flatworm) infections

• bile duct infections or chronic inflammation

• ulcerative colitis

• exposure to chemicals used in industries such as aircraft manufacturing

• rare conditions, such as primary sclerosing cholangitis, hepatitis, Lynch syndrome, or biliary papillomatosis

How is cholangiocarcinoma diagnosed?

Your doctor will perform a physical examination and may take blood samples. Blood tests can check how well your liver is functioning and can be used to look for substances called tumor markers. Levels of tumor markers might rise in people with cholangiocarcinoma.

You may also need imaging scans such as an ultrasound, CT scan, and MRI scan. These provide pictures of your bile ducts and the areas around them and can reveal tumors.

Imaging scans can also help to guide your surgeon’s movements to remove a sample of tissue in what is called an imaging-assisted biopsy.

A procedure known as an endoscopic retrograde cholangiopancreatography (ERCP) is sometimes done. During ERCP, your surgeon passes a long tube with a camera down your throat and into the part of your gut where the bile ducts open. Your surgeon may inject dye into the bile ducts. This helps the ducts show up clearly on an X-ray, revealing any blockages.

In some cases, they’ll also pass a probe that takes ultrasound pictures in the area of your bile ducts. This is called an endoscopic ultrasound scan.

In the test known as percutaneous transhepatic cholangiography (PTC), your doctor takes X-rays after injecting dye into your liver and bile ducts. In this case, they inject the dye straight into your liver through the skin of your abdomen.

How is cholangiocarcinoma treated?

Your treatment will vary according to the location and size of your tumor, whether it has spread (metastasized), and the state of your overall health.


Surgical treatment is the only option that offers a cure, especially if your cancer has been caught early and hasn’t spread beyond your liver or bile ducts. Sometimes, if a tumor is still confined to the bile ducts, you may only need to have the ducts removed. If the cancer has spread beyond the ducts and into your liver, part or all of the liver may have to be removed. If your entire liver must be removed, you’ll need a liver transplant to replace it.

If your cancer has invaded nearby organs, a Whipple procedure may be carried out. In this procedure, your surgeon removes:

• the bile ducts

• the gallbladder

• the pancreas

• sections of your stomach and gut

Even if your cancer can’t be cured, you can have surgery to treat the blocked bile ducts and relieve some of your symptoms. Typically, the surgeon either inserts a tube to hold the duct open or creates a bypass. This can help to treat your jaundice. A blocked section of the gut can also be treated surgically.

You might need to receive chemotherapy or radiation treatments following your surgery.

What is the long-term outlook for people with cholangiocarcinoma?

If it’s possible to remove your tumor completely, you have a chance of being cured. Your outlook is generally better if the tumor isn’t in your liver.

Many people aren’t eligible for surgery that removes the tumor by removing all or part of the liver or bile duct. This can be because the cancer is too advanced, has already metastasized, or is in an inoperable location.