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What is cholangiocarcinoma (CCA)?

What is CCA
Cholangiocarcinoma is cancer that forms in the bile ducts
  • Cholangio = bile duct
  • Carcinoma = a form of cancer
Icône d'informations You might also hear cholangiocarcinoma referred to as bile duct cancer or CCA for short
What are the bile ducts?
  • Bile ducts are the thin tubes that connect your liver, gallbladder and small intestine. Their job is to carry a digestive fluid called bile
  • Bile is made in your liver, is stored in the gallbladder (a small pouch that sits under the liver) and helps to break down fats in the small intestine.1 The bile ducts are also called the biliary system, biliary tree or biliary tract
Diagram of stomach, intestine, gall bladder, liver and bile duct
How common is CCA?
CCA overall

CCA is a rare cancer.2,3 The number of people diagnosed with CCA varies between countries.2-4 In most countries, fewer than 6 in 100,000 people each year will be diagnosed with CCA2,3,5

CCA globe Asia

The lowest rates have been reported in Europe, Australia and the US, and the highest rates in Southeast Asia2,5,6

CCA globe common

CCA has become more common in most countries worldwide, including those in Western Europe, during the last 20–30 years4-6

What are the different types of CCA?
Doctors divide CCA into three different types, depending on where it occurs in relation to the liver:3,5–7
  • Intrahepatic (iCCA) means the cancer affects the bile ducts inside the liver
  • Perihilar (or hilar) (pCCA) means the cancer occurs immediately outside the liver, where the left and right bile ducts join together
  • Distal CCA (dCCA) means the cancer is located in the bile ducts outside the liver, nearer to the intestine
Diagram of liver and bile duct
Figure adapted from Blechacz B. Gut Liver. 2017;11:13–26.
Together, perihilar and distal CCA are sometimes called extrahepatic CCA (eCCA) because theyoccur outside the liver3,6
Perihilar CCA is the most common type of CCA
(50–60% of cases)
Followed by distal (20–30%)
…and intrahepatic (10–20%)3
50-60 Percent Cases
Perihilar CCA is the most common type of CCA
(50–60% of cases)
20-30 Percent Cases
Followed by distal (20–30%)
10-20 Percent Cases
…and intrahepatic (10–20%)3
Proportions are based on USA population data.
Icône d'informations
Each subtype of CCA has different features.7 Understanding the type of CCA you have will help your doctor decide how to treat your cancer
What are the risk factors for CCA?
  • Having a risk factor does not necessarily mean this was the cause of your CCA, and not all risk factors have the same effect. Most people will develop CCA without having an identifiable risk factor2,3,8,9
Over 50

CCA is most often diagnosed in people over the age of 50 years (>65 years in Western countries).2,5 It is slightly more common (approximately 1.5 times) in men than in women4,9


There are many other risk factors for CCA. Some of these risk factors are more established than others2

Some risk factors are common and others are rare, depending on the type of CCA and region of the world2,3,5,6,8-12

Some of the risk factors, such as smoking or being overweight, are not unique to CCA

  • Bile duct cysts and Caroli disease
    These are rare inherited conditions in which the bile ducts are dilated
  • Primary sclerosing cholangitis
    This is a rare condition in which the body’s own immune system attacks the bile ducts, causing them to become inflamed and blocked
  • Blockage of the bile ducts by gallstones (hepatolithiasis)
    Gallstones are hardened deposits of the digestive fluid called bile that develop in the gallbladder and may become trapped in the bile ducts
  • Infection by a virus that inflames the liver (viral hepatitis)
  • Scarring of the liver (cirrhosis)
  • In some areas of the world, such as certain regions of Southeast Asia, it is possible to have parasitic flatworms by eating undercooked fish
  • Inflammation of the intestines ( Crohn’s disease and ulcerative colitis)
  • Inflammation of the pancreas (chronic pancreatitis)
  • Ulcers in the lining of the stomach or the upper part of the small intestine
  • Excessive alcohol consumption (>10 units/day [80 g]) or tobacco smoking
  • Being overweight or obese
  • High blood sugar levels (type 2 diabetes)
  • Build-up of fat in the liver (non-alcoholic fatty liver disease)
  • Thorotrast (a banned substance previously used in medical imaging)
  • 1,2‐dichloropropane (a banned organic solvent used in printing)
  • Asbestos (a fibrous mineral widely used in construction until the late 1990s)
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