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
- Mortality of cholangiocarcinoma is slightly higher in men (1.9/100.000) than in women (1.5/100.000).[5]
Age-standardized mortality rates from intrahepatic (IC) and extrahepatic (EC) cholangiocarcinoma for men and women, by country are illustrated in the following table:
| 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
- Cholangiocarcinoma is more common in males than in females (possibly due to the higher rate of primary sclerosing cholangitis, a major risk factor, in men).[8]
Age
- Patients of all age groups may develop cholangiocarcinoma.
- The average age of the patients at the time of cholangiocarcinoma diagnosis is 70-80 years, except in patients with bile duct cystic disorders, which usually develop cholangiocarcinoma much earlier, between 30 and 40 years.[5]
- The incidence of cholangiocarcinoma increases with age.[9]
References
- ↑ Vauthey J, Blumgart L (1994). "Recent advances in the management of cholangiocarcinomas". Semin Liver Dis. 14 (2): 109–14. PMID 8047893.
- ↑ 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.
- ↑ 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.
- ↑ Vauthey J, Blumgart L (1994). "Recent advances in the management of cholangiocarcinomas". Semin Liver Dis. 14 (2): 109–14. PMID 8047893.
- ↑ 5.0 5.1 5.2 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.
- ↑ Rosen C, Nagorney D, Wiesner R, Coffey R, LaRusso N (1991). "Cholangiocarcinoma complicating primary sclerosing cholangitis". Ann Surg. 213 (1): 21–5. PMID 1845927.
- ↑ 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.
- ↑ Cholangiocarcinoma. Radiopaedia. http://radiopaedia.org/articles/cholangiocarcinoma
- ↑ 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.