Key Points about Cholangiocarcinoma

  • Cholangiocarcinoma is cancer that affects the bile ducts – tubes that connect the gallbladder to the small intestine.
  • Doctors use imaging tests, biopsy, liver function tests and physical exams to diagnose cholangiocarcinoma.
  • Treatment for cholangiocarcinoma may include surgery, liver transplant, chemotherapy, targeted therapy and/or radiation therapy.


Bile ducts are thin tubes that carry bile (digestive fluid) from your gallbladder to your small intestine. Cholangiocarcinoma is a cancer that begins in the bile ducts. There are three main types of cholangiocarcinoma or bile duct cancer:

  • Distal cholangiocarcinoma – this type occurs in the area of the bile duct closest to the small intestine. 
  • Hilar cholangiocarcinoma – this type occurs in the bile ducts closest to the liver.
  • Intrahepatic cholangiocarcinoma – this type occurs in the bile ducts located inside the liver.

Cholangiocarcinoma causes

Cholangiocarcinoma occurs when the cells of the bile ducts develop mutations (changes) to their DNA, and then those abnormal cells multiply out of control.

Cholangiocarcinoma risk factors

Factors that can increase your risk for developing cholangiocarcinoma include:

  • Being a smoker
  • Being older than age 50
  • Having a liver parasite (common in areas of southeast Asia)
  • Having chronic liver disease
  • Having congenital (present at birth) bile duct problems
  • Having cystic fibrosis 
  • Having type 1 or type 2 diabetes 
  • Having Lynch syndrome
  • Having primary sclerosing cholangitis (a condition that affects the bile ducts)

Cholangiocarcinoma symptoms

Signs and symptoms of this condition can include:

  • Dark-colored urine
  • Fatigue
  • Fever
  • Jaundice (yellowing of the whites of the eyes and skin)
  • Night sweats
  • Pain on the right side of your abdomen, just below the ribs
  • Unintentional weight loss
  • Very itchy skin
  • White-colored stool

Cholangiocarcinoma diagnosis

Your oncologist may use one or more of the following tests to diagnose this condition:

  • Physical exam – your doctor will perform a complete physical exam, including asking questions about your health history, symptoms and related risk factors. 
  • Biopsy – in this test, your doctor removes a biopsy (small sample) from the suspicious area. This sample is sent to the laboratory, where a specialist closely checks the biopsy for cancer. 
  • Blood test – your doctor sends a sample of your blood to the laboratory for close analysis. Lab technicians can determine if certain tumor markers are present in your blood.
  • Computerized tomography (CT) scan – this type of imaging test provides a 3D image of the inside of the body that your doctor can use to determine the size and location of the cancer.
  • Endoscopic retrograde cholangiopancreatography (ERCP) – during this test, a thin, flexible tube with a camera on the end is passed through your throat and into your digestive tract to your small intestine. Your doctor can use specialized imaging equipment to closely examine the inside of your body during ERCP.
  • Liver function test – these specialized blood tests can help your specialist determine how well your liver is working.
  • Magnetic resonance imaging (MRI) – this type of imaging test uses high-powered magnets to create detailed images of the inside of your body. Your doctor can closely examine these images to determine the size and location of the cancer.

Cholangiocarcinoma treatments

Depending on your personal health history, the extent of the cholangiocarcinoma and other factors, your oncologist may recommend one or more of the following treatment options:

  • Surgery – you may need to undergo surgery to remove the cancerous area. Your surgeon will work to preserve as much surrounding healthy tissue as possible.
  • Liver transplant – if you have hilar cholangiocarcinoma, you may undergo a liver transplant. During this procedure, your surgeon removes your diseased liver and replaces it with a healthy donor liver. 
  • Chemotherapy – you may need to also undergo chemotherapy to destroy any cancerous cells that couldn’t be removed surgically. During this treatment, medication is used to destroy cancerous cells. Chemotherapy can be taken via an oral (by mouth) pill or intravenously (through a vein).
  • Radiation therapy – this treatment uses high-powered energy beams to destroy cancerous cells. You may need to undergo radiation therapy to destroy any cancerous cells that couldn’t be removed surgically.
  • Targeted drug therapy – this treatment involves taking medications that target specific weaknesses of the cancerous cells, working to destroy them.

When should I seek care?

If you experience any of these symptoms, start by voicing your concerns and symptoms to your primary care provider. From there, your doctor may suggest seeing an oncologist for more specialized treatment.

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