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Epidemiology

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Hilar Cholangiocarcinoma

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Abstract

Cholangiocarcinoma is the second most common primary hepatic malignancy after hepatocellular cancer. It accounts for approximately 10–25 % of all hepatobiliary malignancies. Hilar cholangiocarcinoma is identified based on anatomic location, but the epidemiology is typically aggregated with all cholangiocarcinomas. There are considerable geographic and demographic variations in the incidence of cholangiocarcinoma. These variations are related to risk factors, some of which are established (parasitic infections, primary sclerosing cholangitis, biliary-duct cysts, hepatolithiasis, toxins) and others that are less-established or potential risk factors (inflammatory bowel disease, hepatitis C virus, hepatitis B virus, cirrhosis, diabetes, obesity, alcohol, smoking, host genetic polymorphisms).

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References

  1. Gatto M, Bragazzi MC, Semeraro R, et al. Cholangiocarcinoma: update and future perspectives. Dig Liver Dis. 2010;42(4):253–60.

    Article  PubMed  CAS  Google Scholar 

  2. Patel T. Cholangiocarcinoma. Nat Clin Pract Gastroenterol Hepatol. 2006;3(1):33–42.

    Article  PubMed  Google Scholar 

  3. Shaib Y, El-Serag HB. The epidemiology of cholangiocarcinoma. Semin Liver Dis. 2004;24(2):115–25.

    Article  PubMed  Google Scholar 

  4. Blechacz BR, Gores GJ. Cholangiocarcinoma. Clin Liver Dis. 2008;12(1):131–50.

    Article  PubMed  Google Scholar 

  5. Sripa B, Pairojkul C. Cholangiocarcinoma: lessons from Thailand. Curr Opin Gastroenterol. 2008;24(3):349–56.

    Article  PubMed  Google Scholar 

  6. Chamberlain RS, Blumgart LH. Hilar cholangiocarcinoma: a review and commentary. Ann Surg Oncol. 2000;7(1):55–66.

    Article  PubMed  CAS  Google Scholar 

  7. Ebata T, Kamiya J, Nishio H, et al. The concept of perihilar cholangiocarcinoma is valid. Br J Surg. 2009;96(8):926–34.

    Article  PubMed  CAS  Google Scholar 

  8. Nakeeb A, Pitt HA, Sohn TA, et al. Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg. 1996;224(4):463–73.

    Article  PubMed  CAS  Google Scholar 

  9. Alvaro D, Crocetti E, Ferretti S, et al. Descriptive epidemiology of cholangiocarcinoma in Italy. Dig Liver Dis. 2010;42(7):490–5.

    Article  PubMed  Google Scholar 

  10. El-Serag HB, Engels EA, Landgren O, et al. Risk of hepatobiliary and pancreatic cancers after hepatitis C virus infection: a population-based study of U.S. veterans. Hepatology. 2009;49(1):116–23.

    Article  PubMed  Google Scholar 

  11. Erichsen R, Jepsen P, Vilstrup H, et al. Incidence and prognosis of cholangiocarcinoma in Danish patients with and without inflammatory bowel disease: a national cohort study, 1978–2003. Eur J Epidemiol. 2009;24(9):513–20.

    Article  PubMed  Google Scholar 

  12. Hoblinger A, Grunhage F, Sauerbruch T, et al. Association of the c.3972C>T variant of the multidrug resistance-associated protein 2 Gene (MRP2/ABCC2) with susceptibility to bile duct cancer. Digestion. 2009;80(1):36–9.

    Article  PubMed  CAS  Google Scholar 

  13. Kobayashi M, Ikeda K, Saitoh S, et al. Incidence of primary cholangiocellular carcinoma of the liver in Japanese patients with hepatitis C virus-related cirrhosis. Cancer. 2000;88(11):2471–7.

    Article  PubMed  CAS  Google Scholar 

  14. Kobayashi S, Asano T, Yamasaki M, et al. Risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction. Surgery. 1999;126(5):939–44.

    Article  PubMed  CAS  Google Scholar 

  15. Rosen CB, Nagorney DM, Wiesner RH, et al. Cholangiocarcinoma complicating primary sclerosing cholangitis. Ann Surg. 1991;213(1):21–5.

    Article  PubMed  CAS  Google Scholar 

  16. Shaib YH, El-Serag HB, Davila JA, et al. Risk factors of intrahepatic cholangiocarcinoma in the United States: a case-control study. Gastroenterology. 2005;128(3):620–6.

    Article  PubMed  Google Scholar 

  17. Shaib YH, El-Serag HB, Nooka AK, et al. Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: a hospital-based case-control study. Am J Gastroenterol. 2007;102(5):1016–21.

    Article  PubMed  Google Scholar 

  18. Shin HR, Oh JK, Lim MK, et al. Descriptive epidemiology of cholangiocarcinoma and clonorchiasis in Korea. J Korean Med Sci. 2010;25(7):1011–6.

    Article  PubMed  Google Scholar 

  19. Watanabe Y, Toki A, Todani T. Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg. 1999;6(3):207–12.

    Article  PubMed  CAS  Google Scholar 

  20. Welzel TM, Graubard BI, El-Serag HB, et al. Risk factors for intrahepatic and extrahepatic cholangiocarcinoma in the United States: a population-based case-control study. Clin Gastroenterol Hepatol. 2007;5(10):1221–8.

    Article  PubMed  Google Scholar 

  21. Surveillance, Epidemiology, and End Results (SEER) Program. Research Data (1973–2007), National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch. 2011. www.seer.cancer.gov. Released April 2010, based on the November 2009 submission.

  22. Khan SA, Toledano MB, Taylor-Robinson SD. Epidemiology, risk factors, and pathogenesis of cholangiocarcinoma. HPB (Oxford). 2008;10(2):77–82.

    Article  CAS  Google Scholar 

  23. Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology. 2001;33(6):1353–7.

    Article  PubMed  CAS  Google Scholar 

  24. Patel T. Worldwide trends in mortality from biliary tract malignancies. BMC Cancer. 2002;2:10.

    Article  PubMed  Google Scholar 

  25. Taylor-Robinson SD, Toledano MB, Arora S, et al. Increase in mortality rates from intrahepatic cholangiocarcinoma in England and Wales 1968–1998. Gut. 2001;48(6):816–20.

    Article  PubMed  CAS  Google Scholar 

  26. Welzel TM, McGlynn KA, Hsing AW, et al. Impact of classification of hilar cholangiocarcinomas (Klatskin tumors) on the incidence of intra- and extrahepatic cholangiocarcinoma in the United States. J Natl Cancer Inst. 2006;98(12):873–5.

    Article  PubMed  Google Scholar 

  27. Kaewpitoon N, Kaewpitoon SJ, Pengsaa P, et al. Opisthorchis viverrini: the carcinogenic human liver fluke. World J Gastroenterol. 2008;14(5):666–74.

    Article  PubMed  CAS  Google Scholar 

  28. Upatham ES, Viyanant V. Opisthorchis viverrini and ­opisthorchiasis: a historical review and future perspective. Acta Trop. 2003;88(3):171–6.

    Article  PubMed  CAS  Google Scholar 

  29. Parkin DM, Srivatanakul P, Khlat M, et al. Liver cancer in Thailand I. A case-control study of cholangiocarcinoma. Int J Cancer. 1991;48(3):323–8.

    Article  PubMed  CAS  Google Scholar 

  30. Honjo S, Srivatanakul P, Sriplung H, et al. Genetic and environmental determinants of risk for cholangiocarcinoma via Opisthorchis viverrini in a densely infested area in Nakhon Phanom, northeast Thailand. Int J Cancer. 2005;117(5):854–60.

    Article  PubMed  CAS  Google Scholar 

  31. Shin HR, Lee CU, Park HJ, et al. Hepatitis B and C virus, Clonorchis sinensis for the risk of liver cancer: a case-control study in Pusan, Korea. Int J Epidemiol. 1996;25(5):933–40.

    Article  PubMed  CAS  Google Scholar 

  32. Soreide K, Korner H, Havnen J, et al. Bile duct cysts in adults. Br J Surg. 2004;91(12):1538–48.

    Article  PubMed  CAS  Google Scholar 

  33. Edil BH, Cameron JL, Reddy S, et al. Choledochal cyst disease in children and adults: a 30-year single-institution experience. J Am Coll Surg. 2008;206(5):1000–5.

    Article  PubMed  Google Scholar 

  34. Mabrut JY, Bozio G, Hubert C, et al. Management of congenital bile duct cysts. Dig Surg. 2010;27(1):12–8.

    Article  PubMed  Google Scholar 

  35. Yamaguchi M. Congenital choledochal cyst. Analysis of 1433 patients in the Japanese literature. Am J Surg. 1980;140(5):653–7.

    Article  PubMed  CAS  Google Scholar 

  36. Todani T, Watanabe Y, Toki A, et al. Carcinoma related to ­choledochal cysts with internal drainage operations. Surg Gynecol Obstet. 1987;164(1):61–4.

    PubMed  CAS  Google Scholar 

  37. Lee TY, Lee SS, Jung SW, et al. Hepatitis B virus infection and intrahepatic cholangiocarcinoma in Korea: a case-control study. Am J Gastroenterol. 2008;103(7):1716–20.

    Article  PubMed  Google Scholar 

  38. Voyles CR, Smadja C, Shands WC, et al. Carcinoma in choledochal cysts. Age-related incidence. Arch Surg. 1983;118(8):986–8.

    Article  PubMed  CAS  Google Scholar 

  39. Claessen MM, Vleggaar FP, Tytgat KM, et al. High lifetime risk of cancer in primary sclerosing cholangitis. J Hepatol. 2009;50(1):158–64.

    Article  PubMed  Google Scholar 

  40. LaRusso NF, Shneider BL, Black D, et al. Primary sclerosing cholangitis: summary of a workshop. Hepatology. 2006;44(3):746–64.

    Article  PubMed  Google Scholar 

  41. Burak K, Angulo P, Pasha TM, et al. Incidence and risk factors for cholangiocarcinoma in primary sclerosing cholangitis. Am J Gastroenterol. 2004;99(3):523–6.

    Article  PubMed  Google Scholar 

  42. Boberg KM, Bergquist A, Mitchell S, et al. Cholangiocarcinoma in primary sclerosing cholangitis: risk factors and clinical presentation. Scand J Gastroenterol. 2002;37(10):1205–11.

    Article  PubMed  CAS  Google Scholar 

  43. Kornfeld D, Ekbom A, Ihre T. Survival and risk of ­cholangiocarcinoma in patients with primary sclerosing cholangitis. A population-based study. Scand J Gastroenterol. 1997;32(10):1042–5.

    Article  PubMed  CAS  Google Scholar 

  44. Kubo S, Kinoshita H, Hirohashi K, et al. Hepatolithiasis associated with cholangiocarcinoma. World J Surg. 1995;19(4):637–41.

    Article  PubMed  CAS  Google Scholar 

  45. Lesurtel M, Regimbeau JM, Farges O, et al. Intrahepatic ­cholangiocarcinoma and hepatolithiasis: an unusual association in Western countries. Eur J Gastroenterol Hepatol. 2002;14(9):1025–7.

    Article  PubMed  CAS  Google Scholar 

  46. Huang MH, Chen CH, Yen CM, et al. Relation of hepatolithiasis to helminthic infestation. J Gastroenterol Hepatol. 2005;20(1):141–6.

    Article  PubMed  Google Scholar 

  47. Zhou YM, Yin ZF, Yang JM, et al. Risk factors for intrahepatic ­cholangiocarcinoma: a case-control study in China. World J Gastroenterol. 2008;14(4):632–5.

    Article  PubMed  Google Scholar 

  48. Donato F, Gelatti U, Tagger A, et al. Intrahepatic cholangiocarcinoma and hepatitis C and B virus infection, alcohol intake, and hepatolithiasis: a case-control study in Italy. Cancer Causes Control. 2001;12(10):959–64.

    Article  PubMed  CAS  Google Scholar 

  49. Andersson M, Vyberg M, Visfeldt J, et al. Primary liver tumors among Danish patients exposed to Thorotrast. Radiat Res. 1994;137(2):262–73.

    Article  PubMed  CAS  Google Scholar 

  50. Kato I, Kido C. Increased risk of death in thorotrast-exposed patients during the late follow-up period. Jpn J Cancer Res. 1987;78(11):1187–92.

    PubMed  CAS  Google Scholar 

  51. Lipshutz GS, Brennan TV, Warren RS. Thorotrast-induced liver neoplasia: a collective review. J Am Coll Surg. 2002;195(5):713–8.

    Article  PubMed  Google Scholar 

  52. van Kaick G, Wesch H, Luhrs H, et al. Neoplastic diseases induced by chronic alpha-irradiation – epidemiological, biophysical and clinical results of the German Thorotrast Study. J Radiat Res (Tokyo). 1991;32 Suppl 2Suppl 2:20–33.

    Article  Google Scholar 

  53. Yamamoto S, Kubo S, Hai S, et al. Hepatitis C virus infection as a likely etiology of intrahepatic cholangiocarcinoma. Cancer Sci. 2004;95(7):592–5.

    Article  PubMed  CAS  Google Scholar 

  54. Sorensen HT, Friis S, Olsen JH, et al. Risk of liver and other types of cancer in patients with cirrhosis: a nationwide cohort study in Denmark. Hepatology. 1998;28(4):921–5.

    Article  PubMed  CAS  Google Scholar 

  55. Songsivilai S, Dharakul T, Kanistanon D. Hepatitis C virus genotypes in patients with hepatocellular carcinoma and cholangiocarcinoma in Thailand. Trans R Soc Trop Med Hyg. 1996;90(5):505–7.

    Article  PubMed  CAS  Google Scholar 

  56. Welzel TM, Mellemkjaer L, Gloria G, et al. Risk factors for intrahepatic cholangiocarcinoma in a low-risk population: a nationwide case-control study. Int J Cancer. 2007;120(3):638–41.

    Article  PubMed  CAS  Google Scholar 

  57. Grainge MJ, West J, Solaymani-Dodaran M, et al. The antecedents of biliary cancer: a primary care case-control study in the United Kingdom. Br J Cancer. 2009;100(1):178–80.

    Article  PubMed  CAS  Google Scholar 

  58. Huang WY, Gao YT, Rashid A, et al. Selected base excision repair gene polymorphisms and susceptibility to biliary tract cancer and biliary stones: a population-based case-control study in China. Carcinogenesis. 2008;29(1):100–5.

    Article  PubMed  CAS  Google Scholar 

  59. Ko KH, Kim NK, Yim DJ, et al. Polymorphisms of 5,10-­methylenetetrahydrofolate reductase (MTHFR C677T) and thymidylate synthase enhancer region (TSER) as a risk factor of cholangiocarcinoma in a Korean population. Anticancer Res. 2006;26(6B):4229–33.

    PubMed  CAS  Google Scholar 

  60. Marahatta SB, Punyarit P, Bhudisawasdi V, et al. Polymorphism of glutathione S-transferase omega gene and risk of cancer. Cancer Lett. 2006;236(2):276–81.

    Article  PubMed  CAS  Google Scholar 

  61. Melum E, Karlsen TH, Schrumpf E, et al. Cholangiocarcinoma in primary sclerosing cholangitis is associated with NKG2D polymorphisms. Hepatology. 2008;47(1):90–6.

    Article  PubMed  CAS  Google Scholar 

  62. Prawan A, Kukongviriyapan V, Tassaneeyakul W, et al. Association between genetic polymorphisms of CYP1A2, arylamine N-acetyltransferase 1 and 2 and susceptibility to cholangiocarcinoma. Eur J Cancer Prev. 2005;14(3):245–50.

    Article  PubMed  CAS  Google Scholar 

  63. Sakoda LC, Gao YT, Chen BE, et al. Prostaglandin-endoperoxide synthase 2 (PTGS2) gene polymorphisms and risk of biliary tract cancer and gallstones: a population-based study in Shanghai, China. Carcinogenesis. 2006;27(6):1251–6.

    Article  PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Section of Gastroenterology and Hepatology, Baylor College of Medicine, Houston, TX, USA

    G. L. Tyson MD

  2. Section of Gastroenterology and Hepatology, Michael E. DeBakey Veterans Affairs Medical Center, Baylor College of Medicine, Houston, TX, USA

    S. Keihanian MD

  3. Gastroenterology and Hepatology Section, Houston AV Medical Center and Baylor College of Medicine, Houston, TX, USA

    H. B. El-Serag MD, MPH

Authors
  1. G. L. Tyson MD
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  2. S. Keihanian MD
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  3. H. B. El-Serag MD, MPH
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Correspondence to H. B. El-Serag MD, MPH .

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Editors and Affiliations

  1. , Faculty of Medicine, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong, N/A, Hong Kong SAR

    W.Y. Lau

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Tyson, G.L., Keihanian, S., El-Serag, H.B. (2013). Epidemiology. In: Lau, W. (eds) Hilar Cholangiocarcinoma. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-6473-6_2

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  • DOI: https://doi.org/10.1007/978-94-007-6473-6_2

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