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Gallenblasen- und Gallengangskarzinom

Biologie und Pathologie

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Zusammenfassung

Adenokarzinome der Gallenblase und Gallengänge entstehen durch maligne Transformation des Epithels im Sinne einer Dysplasie-Karzinom-Sequenz. Dabei nehmen die intrahepatischen Cholangiokarzinome ihren Ausgang in den kleinen, portalen, intralobulären Gallengängen. Extrahepatische Gallengangskarzinome entstehen in Ductus hepaticus, Ductus cysticus oder Ductus choledochus. Bei primärem Ursprung in der Bifurkation werden sie Klatskin-Tumoren genannt. Für die intrahepatischen Cholangiokarzinome gilt die UICC-TNM-Klassifikation der Lebertumoren, die extrahepatischen besitzen eine eigene UICC-TNM-Klassifikation. Ätiopathogenetisch sind Faktoren relevant, die mit einer chronischen Entzündungsreaktion einhergehen, wie parasitäre Erkrankungen (Schistosomisasis), Colitis ulcerosa mit konsekutiver primär sklerosierender Cholangitis oder konnatale Leberzysten. Die ätiopathegentische Relevanz von Gallensteinen ist wahrscheinlich. Histologisch handelt es sich um Adenokarzinome; mesenchymale Tumoren oder Melanome sind eine extreme Rarität. Eine ganze Reihe von ursächlichen genetischen Alterationen sind bisher identifiziert worden.

Abstract

Biliary tract cancers are a consequence of a stepwise malignant transformation of the biliary epithelium. Intrahepatic cholangiocarcinomas arises from any portions of the intrahepatic bile duct epithelium: the segmental or area ducts and their finer branches or intrahepatic small bile ducts. Cholangiocarcinoma arising from the hepatic ducts or near their junction are called hilar cholangiocarcinoma or Klatskin tumour, and are considered as extrahepatic lesiona. Cancer of extrahepatic bile ducts may also arise in the Ductus cysticus or choledochus as well as in the gall bladder. For intrahepatic cholangiocarcinoma, the UICC-TNM classification system of malignant liver tumors is applied, differing from the UICC-TNM staging system of extrahepatic bile ducts and gall bladder carcinomas. The cause of carcinomas of the bile ducts remains speculative in most cases. However, chronic inflammation due to sclerosing cholangitis, hepatolithiasis or parasites is associated with carcinogenesis. Histopathologically, the vast majority are adenocarcinomas; mesenchymal tumors and primary melanomas are extremely rare. Different genetic alterations are discussed to be of importance.

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Authors and Affiliations

  1. Institut für Pathologie, Universitätsklinikum Leipzig

    A. Tannapfel & C. Wittekind

  2. Institut für Pathologie, Universitätsklinikum, Liebigstraße 26, 04103, Leipzig

    A. Tannapfel

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  1. A. Tannapfel
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  2. C. Wittekind
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Correspondence to A. Tannapfel.

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Tannapfel, A., Wittekind, C. Gallenblasen- und Gallengangskarzinom. Internist 45, 33–41 (2004). https://doi.org/10.1007/s00108-003-1110-6

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  • DOI: https://doi.org/10.1007/s00108-003-1110-6

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