What is CCA

How common is CCA?

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 CCA^2,3,5

The lowest rates have been reported in Europe, Australia and the US, and the highest rates in Southeast Asia^2,5,6

CCA has become more common in most countries worldwide, including those in Western Europe, during the last 20–30 years^4-6

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 liver^3,6

Perihilar CCA is the most common type of CCA
(50–60% of cases)

Followed by distal (20–30%)…

…and intrahepatic (10–20%)³

Perihilar CCA is the most common type of CCA
(50–60% of cases)

Followed by distal (20–30%)…

…and intrahepatic (10–20%)³

Proportions are based on USA population data.

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 factor^2,3,8,9

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 women^4,9

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 world^2,3,5,6,8-12

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

Other conditions affecting the bile ducts

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

Long-term liver conditions

Infection by a virus that inflames the liver (viral hepatitis)
Scarring of the liver (cirrhosis)

Liver parasites

In some areas of the world, such as certain regions of Southeast Asia, it is possible to have parasitic flatworms by eating undercooked fish

Gastrointestinal/bowel conditions

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

Lifestyle

Excessive alcohol consumption (>10 units/day [80 g]) or tobacco smoking

Metabolic conditions

Being overweight or obese
High blood sugar levels (type 2 diabetes)
Build-up of fat in the liver (non-alcoholic fatty liver disease)

Toxins or chemicals

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)

Learn more about the terms used in this website

More resources

What are the main symptoms of CCA?

How is CCA diagnosed?

How is CCA managed?

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References: 1. Brevini T, et al. J Hepatol. 2020;73:918–32; 2. Bañales JM, et al. Nat Rev Gastroenterol Hepatol. 2016;13:261–80; 3. Bañales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17:557–88; 4. Florio AA, et al. Cancer. 2020;126:2666–78; 5. Blechacz B. Gut Liver. 2017;11:13–26; 6. Valle JW, et al. Ann Oncol. 2016;27(suppl 5):v28–37; 7. Rizvi S, et al. Nat Rev Clin Oncol. 2018;15:95–111. 8. Khan SA, et al. Liver Int. 2019;39(suppl 1):19–31; 9. Tyson GL, El-Serag HB. Hepatology. 2011;54:173–84; 10. Ghidini M, et al. Cancer Manag Res. 2018;11:379–88; 11. Ghouri YA, et al. J Carcinog. 2015;14:1; 12. Sung H, et al. CA Cancer J Clin. 2021;71:209–49.

What is cholangiocarcinoma (CCA)?