What Is the Survival Rate of Cholangiocarcinoma?

Reviewed on 12/30/2020

What Is Cholangiocarcinoma?

Cholangiocarcinoma (bile duct cancer) is a deadly disease. Even when it's detected early, the five year survival rates for people with this cancer is less than 25%.
Cholangiocarcinoma (bile duct cancer) is a deadly disease. Even when it’s detected early, the five year survival rates for people with this cancer is less than 25%.

Cholangiocarcinoma (bile duct cancer) is a rare cancer that occurs when cells in a bile duct grow abnormally and out of control. 

What Are Symptoms of Cholangiocarcinoma?

Cholangiocarcinoma (bile duct cancer) often does not cause symptoms early on and the cancer is often found in later stages. 

When symptoms of cholangiocarcinoma do occur, they may include:

Cholangiocarcinoma is rare. These symptoms are more likely to be caused by something other than cholangiocarcinoma.

What Causes Cholangiocarcinoma?

The exact cause of cholangiocarcinoma (bile duct cancer) is unknown but it is believed to be due to genetic changes. 

Risk factors for developing cholangiocarcinoma include: 

  • Certain diseases of the liver or bile ducts
  • Inflammatory bowel disease
  • Older age: most people diagnosed with cholangiocarcinoma are in their 60s or 70s
  • Ethnicity and geography
    • The risk is highest among Hispanic Americans in the U.S.
    • Worldwide it is more common in Southeast Asia and China
  • Obesity
  • Non-alcoholic fatty liver disease 
  • Exposure to thorium dioxide (Thorotrast), a radioactive contrast agent used for X-rays until the 1950s (it is no longer used)
  • Family history of bile duct cancer
  • Diabetes
  • Alcohol consumption, particularly in people who have liver problems from drinking alcohol
  • Other possible risk factors (though the links are not as clear)

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How Is Cholangiocarcinoma Diagnosed?

In addition to a patient history and physical exam, tests used to diagnose cholangiocarcinoma (bile duct cancer) or to rule out other causes for symptoms include: 

  • Blood tests
    • Tests of liver and gallbladder function
    • Tumor markers
  • Imaging tests
    • Ultrasound
    • Computed tomography (CT) scan
      • CT angiography 
      • CT-guided needle biopsy
    • Magnetic resonance imaging (MRI) scan
      • Magnetic resonance cholangiopancreatography (MRCP)
      • MR angiography (MRA) 
    • Cholangiography
      • Endoscopic retrograde cholangiopancreatography (ERCP)
      • Percutaneous transhepatic cholangiography (PTC)
    • Angiography
  • Other tests

What Is the Treatment for Cholangiocarcinoma?

Treatment for cholangiocarcinoma (bile duct cancer) involves on or more of the following: 

  • Surgery 
    • Resectable tumor (potentially curative surgery in cases where the tumor can be completely removed with wide margins; only a small portion of bile duct cancers are resectable when found)
      • Removal of part of the liver (partial hepatectomy) 
      • Removal of a whole lobe (right or left part) of the liver (hepatic lobectomy)
      • Removal of part of the liver, along with the bile duct, gallbladder, nearby lymph nodes, and sometimes part of the pancreas and small intestine
      • Removal of part of the pancreas and small intestine (Whipple procedure)
    • Unresectable cancers (cancer is too advanced or cannot be fully removed through surgery)
  • Radiation therapy 
    • External beam radiation therapy (EBRT)
    • Three-dimensional conformal radiation therapy (3D-CRT) 
    • Intensity-modulated radiation therapy (IMRT) 
    • Stereotactic body radiotherapy (SBRT) 
    • Chemoradiation 
    • Brachytherapy (internal radiation therapy)
  • Chemotherapy 
  • Targeted therapy 
    • Fibroblast growth factor receptor (FGFR2) inhibitor: pemigatinib (Pemazyre)
  • Immunotherapy 
    • Drugs are being tested in clinical trials to see if they might help treat bile duct cancer
  • Palliative therapy to help control or reduce symptoms 
    • Pain medicines 
    • Drugs to control nausea or itching 
    • Chemotherapy and radiation to relieve problems caused by the tumor(s)
    • Surgery or other treatments to help patients feel better or to help prevent possible problems caused by the cancer 
      • Biliary stent or biliary catheter
      • Biliary bypass
      • Tumor ablation (radiofrequency ablation or cryosurgery)
      • Photodynamic therapy (PDT)
      • Alcohol injection

What Is the Life Expectancy for Cholangiocarcinoma?

Life expectancy for cholangiocarcinoma (bile duct cancer) is often expressed in five-year survival rates, that is, how many people will be alive five years after diagnosis. 

Intrahepatic cholangiocarcinoma (those starting within the liver) five-year survival rates:

  • Localized (no sign that the cancer has spread outside of the bile ducts): 24%
  • Regional (cancer has spread outside the bile ducts to nearby structures or lymph nodes): 7%
  • Distant (cancer has spread to distant parts of the body such as the lungs): 2%

Extrahepatic bile duct cancers (those starting outside the liver) (includes both perihilar and distal bile duct cancers) five-year survival rates:

  • Localized (no sign that the cancer has spread outside of the bile ducts): 15%
  • Regional (cancer has spread outside the bile ducts to nearby structures or lymph nodes): 16%  
  • The 5-year survival for these tumors at the regional stage is slightly better than for the localized stage, although the reason for this is not clear
  • Distant (cancer has spread to distant parts of the body such as the lungs): 6%

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Reviewed on 12/30/2020
References
https://www.cancer.org/cancer/bile-duct-cancer/about.html