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Epidemiologie, molekularbiologische Befunde und Pathohistologie zentraler Gallengangskarzinome

Epidemiology, molecular biology findings and pathohistology of hilar bile duct carcinomas

Der Onkologe volume 12, pages 1191–1198 (2006)Cite this article

Zusammenfassung

Karzinome der extrahepatischen Gallengänge machen weniger als 1% aller malignen Tumoren aus und zeigen eine leicht ansteigende Inzidenz. Sie entstehen durch maligne Transformation des Epithels der extrahepatischen Gallengänge. Ätiopathogenetisch sind Faktoren, die mit einer chronischen Entzündungsreaktion einhergehen, relevant: Colitis ulcerosa mit primär sklerosierender Cholangitis oder Erkrankungen aus dem Formenkreis der Duktalplattenanlagestörungen. Die Karzinome sind im Ductus hepaticus dexter, sinister oder communis, Ductus cysticus oder Ductus choledochus lokalisiert. Bei einer Lokalisation im Bereich der Bifurkation werden sie Klatskin-Tumoren (oder auch zentrale Gallengangskarzinome) genannt. Die Entstehung der Karzinome folgt einem Mehrschrittablauf mit zunehmenden genetischen Dysregulationen und wird beschreibend als intraepitheliale Neoplasie-Karzinom-Sequenz bezeichnet. Eine ganze Reihe von genetischen Alterationen im Rahmen der Karzinomentstehung wurden bisher identifiziert. Es ist noch nicht geklärt, ob sie für die extrahepatischen Gallengangskarzinome typisch sind und welche prognostische Bedeutung sie haben. Bisher gibt es nur punktuelle Hinweise, dass zentrale Gallengangskarzinome im Expressionsmuster verschiedener Gene eher den intrahepatischen Cholangiokarzinomen ähneln als den Karzinomen des distalen Gallengangs. Histologisch zeigen sich in über 90% Adenokarzinome, die nach der WHO klassifiziert werden und verschiedene Ausbreitungsmuster bieten, die wiederum in der UICC-TNM-Klassifikation zusammengefasst werden. Die Prognose der Patienten ist generell schlecht.

Abstract

Cancer of the extrahepatic bile ducts accounts for less than 1% all malignancies with increasing incidence. Although many aetiological factors have been characterized, the cause of extrahepatic bile duct carcinomas remains speculative in most cases. Chronic inflammation due to primary sclerosing cholangitis as well as biliary cysts as a part of ductal plate malformation are associated with carcinogenesis. Biliary tract cancers are a consequence of a stepwise malignant transformation of the biliary epithelium. Cholangiocarcinoma located at the right and left hepatic ducts or near their junction is called hilar (central) cholangiocarcinoma or Klatskin’s tumor and is considered to be an extrahepatic lesion (carcinoma of the extrahepatic bile ducts). Cancer of extrahepatic bile ducts may also arise in the cystic duct or choledochal duct. The development of these carcinomas follows a multistep procedure associated with increasing genetic dysregulation and is called the intraepithelial neoplasia-carcinoma sequence. Several genetic alterations have been identified in these tumours using different techniques. The problem of whether central biliary carcinomas are a separate tumour entity has not yet been solved. From molecular genetic data it can be concluded that they are more similar to intrahepatic cholangiocarcinomas than to carcinomas of the distal bile duct. Histopathologically, the vast majority of biliary cancers are adenocarcinomas, the anatomic extent of which is classified according to the UICC TNM staging system. The prognosis of patients is generally poor.

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  1. Institut für Pathologie, Universitätsklinikum Leipzig, Liebigstraße 26, 04103 , Leipzig, Deutschland

    C. Wittekind

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Wittekind, C. Epidemiologie, molekularbiologische Befunde und Pathohistologie zentraler Gallengangskarzinome. Onkologe 12, 1191–1198 (2006). https://doi.org/10.1007/s00761-006-1135-2

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