The Epidemiology of Cholangiocarcinoma

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
• 2 Jemal A, Murray T, Samuels A et al.. Cancer statistics, 2003.  CA Cancer J Clin. 2003;  53 5-26
• 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
• 4 Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States.  Hepatology. 2001;  33 1353-1357
• 5 Patel T. Worldwide trends in mortality from biliary tract malignancies.  BMC Cancer. 2002;  2 10
• 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
• 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
• 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
• 9 Gumustop B, Aithal G P. Striking trends in the cholangiocarcinoma incidence and mortality in the United States.  Gastroenterology. 2001;  120 A257
• 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
• 11 Okuda K, Nakanuma Y, Miyazaki M. Cholangiocarcinoma: recent progress. Part 1: epidemiology and etiology.  J Gastroenterol Hepatol. 2002;  17 1049-1055
• 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
• 13 Cancer incidence in five continents.  IARC Scientific Publ. 1997;  7 i-xxxiv 1-1240
• 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
• 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
• 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
• 17 Carriaga M T, Henson D E. Liver, gallbladder, extrahepatic bile ducts, and pancreas.  Cancer. 1995;  75(Suppl 1) 171-190
• 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
• 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
• 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
• 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
• 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
• 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
• 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
• 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
• 26 Watanapa P, Watanapa W B. Liver fluke-associated cholangiocarcinoma.  Br J Surg. 2002;  89 962-970
• 27 Abdel-Rahim A Y. Parasitic infections and hepatic neoplasia.  Dig Dis. 2001;  19 288-291
• 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
• 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
• 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
• 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
• 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
• 33 Chen M F. Peripheral cholangiocarcinoma (cholangiocellular carcinoma): clinical features, diagnosis and treatment.  J Gastroenterol Hepatol. 1999;  14 1144-1149
• 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
• 35 Tabata M, Nakayama F. Bacteria and gallstones. Etiological significance.  Dig Dis Sci. 1981;  26 218-224
• 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
• 37 Chijiiwa K, Koga A. Surgical management and long-term follow-up of patients with choledochal cysts.  Am J Surg. 1993;  165 238-242
• 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
• 39 Ohtsuka T, Inoue K, Ohuchida J et al.. Carcinoma arising in choledochocele.  Endoscopy. 2001;  33 614-619
• 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
• 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
• 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
• 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
• 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
• 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
• 46 Wiesner R H, LaRusso N F. Clinicopathologic features of the syndrome of primary sclerosing cholangitis.  Gastroenterology. 1980;  79 200-206