Cholangiocarcinoma epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Epidemiology

  • Cholangiocarcinoma is the second most common type of primary hepatic tumor, with intrahepatic cholangiocarcinomas (ICCs) accounting for 10-20% of primary liver tumors.

Incidence

  • Cholangiocarcinoma is a rare disease.
  • There are significant variations in incidence according to region, with much higher rates seen in southeast Asia.
  • The overall incidence of cholangiocarcinoma in U.S. population is estimated at 1 - 2 cases per 100,000 individuals.[1]
  • Multiple studies have documented a steady increase in the incidence of intrahepatic cholangiocarcinoma over the past several decades. Increases have been observed in North America, Europe, Asia, and Australia.[2] The reasons for the increasing occurrence of cholangiocarcinoma are unclear; improved diagnostic methods may be partially responsible, but the prevalence of potential risk factors for cholangiocarcinoma, such as HIV infection, has also been increasing during this time frame.[3]

Prevalence

  • The prevalence of cholangiocarcinoma is approximately 0.01% to 0.46% per 100,000 individuals.[4]
  • In the United States, the highest prevalence adjusted by age is found in the Hispanic population, whereas the lowest is found in African Americans.[5]
  • There is a higher prevalence of cholangiocarcinoma in Asia, which has been attributed to endemic chronic parasitic infestation.
  • The prevalence of cholangiocarcinoma in patients with primary sclerosing cholangitis may be as high as 30%, based on autopsy studies.[6]

Mortality

Age-standardized mortality rates from intrahepatic (IC) and extrahepatic (EC) cholangiocarcinoma for men and women, by country

Age-standardized mortality rates from intrahepatic (IC) and extrahepatic (EC) cholangiocarcinoma for men and women, by country. Source: Khan et al, 2002.[7]
Country IC (men/women) EC (men/women)
U.S.A. 0.60 / 0.43 0.70 / 0.87
Japan 0.23 / 0.10 5.87 / 5.20
Australia 0.70 / 0.53 0.90 / 1.23
England/Wales 0.83 / 0.63 0.43 / 0.60
Scotland 1.17 / 1.00 0.60 / 0.73
France 0.27 / 0.20 1.20 / 1.37
Italy 0.13 / 0.13 2.10 / 2.60


Gender

Age

  • Patients of all age groups may develop cholangiocarcinoma.
  • The incidence of cholangiocarcinoma increases with age.[9]

References

  1. Vauthey J, Blumgart L (1994). "Recent advances in the management of cholangiocarcinomas". Semin Liver Dis. 14 (2): 109–14. PMID 8047893.
  2. Multiple independent studies have documented a steady increase in the worldwide incidence of cholangiocarcinoma. Some relevant journal articles include:
    • Patel T. "Worldwide trends in mortality from biliary tract malignancies". BMC Cancer. 2: 10. PMID 11991810.
    • Patel T (2001). "Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States". Hepatology. 33 (6): 1353–7. PMID 11391522.
    • Shaib Y, Davila J, McGlynn K, El-Serag H (2004). "Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase?". J Hepatol. 40 (3): 472–7. PMID 15123362.
    • West J, Wood H, Logan R, Quinn M, Aithal G (2006). "Trends in the incidence of primary liver and biliary tract cancers in England and Wales 1971–2001". Br J Cancer. 94 (11): 1751–8. PMID 16736026.
    • Khan S, Taylor-Robinson S, Toledano M, Beck A, Elliott P, Thomas H (2002). "Changing international trends in mortality rates for liver, biliary and pancreatic tumours". J Hepatol. 37 (6): 806–13. PMID 12445422.
    • Welzel T, McGlynn K, Hsing A, O'Brien T, Pfeiffer R (2006). "Impact of classification of hilar cholangiocarcinomas (Klatskin tumors) on the incidence of intra- and extrahepatic cholangiocarcinoma in the United States". J Natl Cancer Inst. 98 (12): 873–5. PMID 16788161.
  3. Shaib Y, El-Serag H, Davila J, Morgan R, McGlynn K (2005). "Risk factors of intrahepatic cholangiocarcinoma in the United States: a case-control study". Gastroenterology. 128 (3): 620–6. PMID 15765398.
  4. Vauthey J, Blumgart L (1994). "Recent advances in the management of cholangiocarcinomas". Semin Liver Dis. 14 (2): 109–14. PMID 8047893.
  5. Macias, Rocio I. R. (2014). "Cholangiocarcinoma: Biology, Clinical Management, and Pharmacological Perspectives". ISRN Hepatology. 2014: 1–13. doi:10.1155/2014/828074. ISSN 2314-4041.
  6. Rosen C, Nagorney D, Wiesner R, Coffey R, LaRusso N (1991). "Cholangiocarcinoma complicating primary sclerosing cholangitis". Ann Surg. 213 (1): 21–5. PMID 1845927.
  7. Khan S, Taylor-Robinson S, Toledano M, Beck A, Elliott P, Thomas H (2002). "Changing international trends in mortality rates for liver, biliary and pancreatic tumours". J Hepatol. 37 (6): 806–13. PMID 12445422.
  8. Cholangiocarcinoma. Radiopaedia. http://radiopaedia.org/articles/cholangiocarcinoma
  9. Henson D, Albores-Saavedra J, Corle D (1992). "Carcinoma of the extrahepatic bile ducts. Histologic types, stage of disease, grade, and survival rates". Cancer. 70 (6): 1498–501. PMID 1516001.

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