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Semin Liver Dis 2004; 24(2): 115-125
DOI: 10.1055/s-2004-828889
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

The Epidemiology of Cholangiocarcinoma

Yasser Shaib1 , Hashem B. El-Serag1 , 2
  • 1Sections of Gastroenterology and Health Services Research, The Houston Veterans Affairs Medical Center and Baylor College of Medicine, Houston, Texas
  • 2Associate Professor of Medicine, Sections of Gastroenterology and Health Services Research, The Houston Veterans Affairs Medical Center and Baylor College of Medicine, Houston, Texas
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Publication History

Publication Date:
11 June 2004 (online)

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The incidence rates of cholangiocarcinoma (CC) vary greatly among different areas of the world, and this variation is related to distribution of risk factors. Intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC) have different epidemiological features. Recent data show that the incidence and mortality rates of ICC have been increasing in several areas around the world. On the other hand, the incidence and mortality rates of ECC have been decreasing. For example, in the United States, the age-adjusted incidence rates of ICC increased by 165% from 0.32 per 100,000 in 1975 to 1979 to 0.85 per 100,000 in 1995 to 1999, whereas ECC declined by 14%. In the meantime, there has been very little improvement in long-term survival, which remains dismal (3.5%). Men are affected 1.5 times more than women are, and Asians are affected almost 2 times more than whites and blacks. There are few well-established risk factors for CC, including primary sclerosing cholangitis, liver fluke infestations, hepatolithiasis, Thorotrast exposure, and choledochal cysts. None of these risk factors can explain the recent increasing trends of ICC in the United States. Some data, however, point to a potential role for chronic liver disease, hepatitis C, and probably hepatitis B infections in the development of ICC.

KEYWORDS

Extrahepatic - survival - risk factors - incidence - SEER

REFERENCES

  • 1 Vauthey J N, Blumgart L H. Recent advances in the management of cholangiocarcinomas.  Semin Liver Dis. 1994;  14 109-114
    Thieme ConnectPubMedSearch in Google Scholar
  • 2 Jemal A, Murray T, Samuels A et al.. Cancer statistics, 2003.  CA Cancer J Clin. 2003;  53 5-26
    CrossrefPubMedSearch in Google Scholar
  • 3 Mouzas I A, Dimoulios P, Vlachonikolis I G et al.. Increasing incidence of cholangiocarcinoma in Crete 1992-2000.  Anticancer Res. 2002;  22 3637-3641
    PubMedSearch in Google Scholar
  • 4 Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States.  Hepatology. 2001;  33 1353-1357
    CrossrefPubMedSearch in Google Scholar
  • 5 Patel T. Worldwide trends in mortality from biliary tract malignancies.  BMC Cancer. 2002;  2 10
    CrossrefPubMedSearch in Google Scholar
  • 6 Taylor-Robinson S D, Toledano M B, Arora S et al.. Increase in mortality rates from intrahepatic cholangiocarcinoma in England and Wales 1968-1998.  Gut. 2001;  48 816-820
    CrossrefPubMedSearch in Google Scholar
  • 7 Khan S A, Taylor-Robinson S D, Toledano M B et al.. Changing international trends in mortality rates for liver, biliary and pancreatic tumours.  J Hepatol. 2002;  37 806-813
    CrossrefPubMedSearch in Google Scholar
  • 8 Shaib Y H, Davila J A, McGlynn K, El-Serag H B. Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase?.  J Hepatol. 2004;  40 472-477
    CrossrefPubMedSearch in Google Scholar
  • 9 Gumustop B, Aithal G P. Striking trends in the cholangiocarcinoma incidence and mortality in the United States.  Gastroenterology. 2001;  120 A257
    PubMedSearch in Google Scholar
  • 10 Strasberg S M, Drebin J A. Tumors of the biliary tree. In: Yamada T Textbook of Gastroenterology. Philadelphia; Lippincott Williams & Wilkins 1999: 2326-2342
    Search in Google Scholar
  • 11 Okuda K, Nakanuma Y, Miyazaki M. Cholangiocarcinoma: recent progress. Part 1: epidemiology and etiology.  J Gastroenterol Hepatol. 2002;  17 1049-1055
    CrossrefPubMedSearch in Google Scholar
  • 12 Altaee M Y, Johnson P J, Farrant J M, Williams R. Etiologic and clinical characteristics of peripheral and hilar cholangiocarcinoma.  Cancer. 1991;  68 2051-2055
    PubMedSearch in Google Scholar
  • 13 Cancer incidence in five continents.  IARC Scientific Publ. 1997;  7 i-xxxiv 1-1240
    PubMedSearch in Google Scholar
  • 14 Parkin D M, Ohshima H, Srivatanakul P, Vatanasapt V. Cholangiocarcinoma: epidemiology, mechanisms of carcinogenesis and prevention.  Cancer Epidemiol Biomarkers Prev. 1993;  2 537-544
    PubMedSearch in Google Scholar
  • 15 de Groen P C, Gores G J, LaRusso N F, Gunderson L L, Nagorney D M. Biliary tract cancers.  N Engl J Med. 1999;  341 1368-1378
    CrossrefPubMedSearch in Google Scholar
  • 16 Strom B L, Hibberd P L, Soper K A, Stolley P D, Nelson W L. International variations in epidemiology of cancers of the extrahepatic biliary tract.  Cancer Res. 1985;  45 5165-5168
    PubMedSearch in Google Scholar
  • 17 Carriaga M T, Henson D E. Liver, gallbladder, extrahepatic bile ducts, and pancreas.  Cancer. 1995;  75(Suppl 1) 171-190
    CrossrefPubMedSearch in Google Scholar
  • 18 Nakanuma Y, Mashiso H, Tadashi T. Clinical and pathologic features of choalngiocarcinoma. In: Okuda K, Tabor E Liver Cancer. New York; Churchill Livingstone 1997: 279-290
    Search in Google Scholar
  • 19 Bergquist A, Glaumann H, Persson B, Broome U. Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: a case-control study.  Hepatology. 1998;  27 311-316
    CrossrefPubMedSearch in Google Scholar
  • 20 Chalasani N, Baluyut A, Ismail A et al.. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study.  Hepatology. 2000;  31 7-11
    CrossrefPubMedSearch in Google Scholar
  • 21 Broome U, Olsson R, Loof L et al.. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.  Gut. 1996;  38 610-615
    PubMedSearch in Google Scholar
  • 22 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 1042-1045
    PubMedSearch in Google Scholar
  • 23 Aadland E, Schrumpf E, Fausa O et al.. Primary sclerosing cholangitis: a long-term follow-up study.  Scand J Gastroenterol. 1987;  22 655-664
    PubMedSearch in Google Scholar
  • 24 Chapman R W, Arborgh B A, Rhodes J M et al.. Primary sclerosing cholangitis: a review of its clinical features, cholangiography, and hepatic histology.  Gut. 1980;  21 870-877
    PubMedSearch in Google Scholar
  • 25 Rosen C B, Nagorney D M, Wiesner R H, Coffey Jr R J, LaRusso N F. Cholangiocarcinoma complicating primary sclerosing cholangitis.  Ann Surg. 1991;  213 21-25
    PubMedSearch in Google Scholar
  • 26 Watanapa P, Watanapa W B. Liver fluke-associated cholangiocarcinoma.  Br J Surg. 2002;  89 962-970
    CrossrefPubMedSearch in Google Scholar
  • 27 Abdel-Rahim A Y. Parasitic infections and hepatic neoplasia.  Dig Dis. 2001;  19 288-291
    CrossrefPubMedSearch in Google Scholar
  • 28 Parkin D M, Srivatanakul P, Khlat M et al.. Liver cancer in Thailand. I. A case-control study of cholangiocarcinoma.  Int J Cancer. 1991;  48 323-328
    CrossrefPubMedSearch in Google Scholar
  • 29 Thamavit W, Bhamarapravati N, Sahaphong S, Vajrasthira S, Angsubhakorn S. Effects of dimethylnitrosamine on induction of cholangiocarcinoma in Opisthorchis viverrini-infected Syrian golden hamsters.  Cancer Res. 1978;  38 4634-4639
    PubMedSearch in Google Scholar
  • 30 Thamavit W, Kongkanuntn R, Tiwawech D, Moore M A. Level of Opisthorchis infestation and carcinogen dose-dependence of cholangiocarcinoma induction in Syrian golden hamsters.  Virchows Arch B Cell Pathol Incl Mol Pathol. 1987;  54 52-58
    PubMedSearch in Google Scholar
  • 31 Shin H R, Lee C U, Park H J 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;  25 933-940
    PubMedSearch in Google Scholar
  • 32 Huang M J, Liaw Y F, Wu C S. Comparison of intravenous radionuclide cholescintigraphy and endoscopic retrograde cholangiography in the diagnosis of intrahepatic gall-stones.  Br J Radiol. 1981;  54 302-306
    PubMedSearch in Google Scholar
  • 33 Chen M F. Peripheral cholangiocarcinoma (cholangiocellular carcinoma): clinical features, diagnosis and treatment.  J Gastroenterol Hepatol. 1999;  14 1144-1149
    CrossrefPubMedSearch in Google Scholar
  • 34 Okuda K, Kubo Y, Okazaki N, Arishima T, Hashimoto M. Clinical aspects of intrahepatic bile duct carcinoma including hilar carcinoma: a study of 57 autopsy-proven cases.  Cancer. 1977;  39 232-246
    CrossrefPubMedSearch in Google Scholar
  • 35 Tabata M, Nakayama F. Bacteria and gallstones. Etiological significance.  Dig Dis Sci. 1981;  26 218-224
    CrossrefPubMedSearch in Google Scholar
  • 36 Simeone D M. Gallbladder and biliary tree: anatomy and structural anomalies. In: Yamada T Textbook of Gastroenterology. Philadelphia; Lippincott Williams & Wilkins 1999: 2244-2257
    Search in Google Scholar
  • 37 Chijiiwa K, Koga A. Surgical management and long-term follow-up of patients with choledochal cysts.  Am J Surg. 1993;  165 238-242
    PubMedSearch in Google Scholar
  • 38 Lipsett P A, Pitt H A, Colombani P M, Boitnott J K, Cameron J L. Choledochal cyst disease. A changing pattern of presentation.  Ann Surg. 1994;  220 644-652
    PubMedSearch in Google Scholar
  • 39 Ohtsuka T, Inoue K, Ohuchida J et al.. Carcinoma arising in choledochocele.  Endoscopy. 2001;  33 614-619
    Thieme ConnectPubMedSearch in Google Scholar
  • 40 Hardell L, Bengtsson N O, Jonsson U, Eriksson S, Larsson L G. Aetiological aspects on primary liver cancer with special regard to alcohol, organic solvents and acute intermittent porphyria-an epidemiological investigation.  Br J Cancer. 1984;  50 389-397
    PubMedSearch in Google Scholar
  • 41 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;  12 959-964
    CrossrefPubMedSearch in Google Scholar
  • 42 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 2471-2477
    CrossrefPubMedSearch in Google Scholar
  • 43 Sorensen H T, Friis S, Olsen J H et al.. Risk of liver and other types of cancer in patients with cirrhosis: a nationwide cohort study in Denmark.  Hepatology. 1998;  28 921-925
    CrossrefPubMedSearch in Google Scholar
  • 44 Yin F, Chen B. Detection of hepatitis C virus RNA sequences in hepatic portal cholangiocarcinoma tissue by reverse transcription polymerase chain reaction.  Chin Med J (Engl). 1998;  111 1068-1070
    PubMedSearch in Google Scholar
  • 45 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;  90 505-507
    CrossrefPubMedSearch in Google Scholar
  • 46 Wiesner R H, LaRusso N F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis.  Gastroenterology. 1980;  79 200-206
    PubMedSearch in Google Scholar

Hashem B El-SeragM.D. 

The Houston Veterans Affairs Medical Center

2002 Holcombe Blvd (152)

Houston, TX 77030

Email: hasheme@bcm.tmc.edu