8160/3 Bile duct adenocarcinoma


Related terms

Cholangiocarcinoma

Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22
ICD10: C22
           

Intrahepatic cholangiocarcinoma (ICC) is an intrahepatic malignancy with biliary epithelial differentiation. ICC can arise in any portion of the intrahepatic biliary tree, from the segmental and area ducts and their major branches to the smallest bile ducts and ductules 1
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



2
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Okuda K, Nakanuma Y, Miyazaki M (2002)
Cholangiocarcinoma: recent progress. Part 1: epidemiology and etiology.
J Gastroenterol Hepatol 17: 1049-55



3
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Patel T (2001)
Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States.
Hepatology 33: 1353-7



. ICC arising from the intrahepatic small bile ducts is often called ?peripheral ICC?, but use of this term is discouraged.

Hilar and perihilar ICC
Cholangiocarcinoma arising from the right and left hepatic ducts at or near their junction is referred to as ?hilar ICC? (often called ?Klatskin tumour?) and is considered to be an extrahepatic lesion
4
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Welzel TM, McGlynn KA, Hsing AW, O'Brien TR, Pfeiffer RM (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: 873-5



, although the differentiation of hilar cholangiocarcinoma from ICC arising from the intrahepatic large bile ducts (perihilar ICC) is usually controversial at advanced stages, and such cases could be included in the definition of perihilar ICC 5
 
Hamilton SR, Aaltonen LA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Digestive System.
3rd Edition
IARC Press: Lyon 2000



6
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Nakeeb A, Pitt HA, Sohn TA, Coleman J, Abrams RA, Piantadosi S, Hruban RH, Lillemoe KD, Yeo CJ, Cameron JL (1996)
Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors.
Ann Surg 224: 463-73; discussion 473-5



. Likewise, it should be kept in mind that the distinction of hilar from perihilar depends on the pathological examination of resected specimens. The uses and meanings of the terms ?hilar? and ?perihilar? therefore may differ between pathologists, surgeons and radiologists.

ICC is a relatively infrequent tumour in most populations. However, the prevalence shows a wide geographic
variation, and the highest incidence is reported in areas of Asia where Opisthorchis viverrini (Thailand and Laos
7
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Lim MK, Ju YH, Franceschi S, Oh JK, Kong HJ, Hwang SS, Park SK, Cho SI, Sohn WM, Kim DI, Yoo KY, Hong ST, Shin HR (2006)
Clonorchis sinensis infection and increasing risk of cholangiocarcinoma in the Republic of Korea.
Am J Trop Med Hyg 75: 93-6



) and Clonorchis sinensis (southern China and the Republic of Korea 8
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Watanapa P, Watanapa WB (2002)
Liver fluke-associated cholangiocarcinoma.
Br J Surg 89: 962-70



) are endemic. The incidence of ICC is also reported to increase in many regions, including non-endemic areas 9
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Khan SA, Taylor-Robinson SD, Toledano MB, Beck A, Elliott P, Thomas HC (2002)
Changing international trends in mortality rates for liver, biliary and pancreatic tumours.
J Hepatol 37: 806-13



10
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Kobayashi M, Ikeda K, Saitoh S, Suzuki F, Tsubota A, Suzuki Y, Arase Y, Murashima N, Chayama K, Kumada H (2000)
Incidence of primary cholangiocellular carcinoma of the liver in japanese patients with hepatitis C virus-related cirrhosis.
Cancer 88: 2471-7



11
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Patel T (2001)
Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States.
Hepatology 33: 1353-7



12
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Shaib YH, Davila JA, McGlynn K, El-Serag HB (2004)
Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase?
J Hepatol 40: 472-7



13
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Welzel TM, McGlynn KA, Hsing AW, O'Brien TR, Pfeiffer RM (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: 873-5



. The etiology of most cases remains obscure, some cases are associated with prior biliary or hepatic disease and a number of other risk factors 14
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Chapman RW (1999)
Risk factors for biliary tract carcinogenesis.
Ann Oncol 10 Suppl 4: 308-11



. ICC is associated with a high rate of fatality because of early invasion, widespread metastasis, and lack of effective therapeutic modalities15
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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