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Cholangiokarzinome – Übersicht zur aktuellen anatomischen, histomorphologischen und morphomolekularen Klassifikation

Cholangiocarcinoma—diagnosis, classification, and molecular alterations

  • Schwerpunkt: Leberpathologie
  • Published:
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Zusammenfassung

Hintergrund

Unter dem Begriff Cholangiokarzinom werden diverse maligne epitheliale Neoplasien unterschiedlicher Differenzierung, Ätiologie und Pathogenese zusammengefasst.

Ziel der Arbeit

In dieser Arbeit sollen die Ätiologie, Pathogenese, Diagnostik, Klassifikation und molekulare Alterationen intra- und extrahepatischer Cholangiokarzinome dargestellt werden.

Material und Methoden

Grundlage hierfür sind die derzeit verfügbare Fachliteratur sowie eigene Erkenntnisse.

Ergebnisse und Diskussion

Cholangiokarzinome sind morphologisch und molekular vielgestaltige maligne epitheliale Neoplasien, die ubiquitär im Gallengangsystem entstehen können. Da histologisch und immunhistochemisch breite Überlappungen zu den häufigen metastatischen Absiedelungen in der Leber bestehen, sollte die definitive Diagnose nur im klinischen Kontext gestellt werden. Nach makroanatomischen Kriterien werden Cholangiokarzinome in intrahepatische (iCCA), perihiläre (pCCA) und distale (dCCA) Tumore subklassifiziert. Wie sich durch neuere Untersuchungen abzeichnet, bestehen insbesondere zwischen den iCCA und den extrahepatischen CCA (eCCA, diese umfassen pCCA und dCCA) deutliche molekulare Unterschiede. Zudem konnten mittlerweile morphologisch gut charakterisierte Vorläuferläsionen identifiziert werden, die sich in verschiedenen Häufigkeiten nicht nur der anatomischen Klassifikation, sondern auch distinkten Ätiologien zuordnen lassen. Eine exakte Klassifikation der CCA ist somit essenziell, vor allem im Hinblick auf die Entwicklung innovativer, zielgerichteter Therapieansätze.

Abstract

Background

The entity cholangiocarcinoma comprises various malignant epithelial neoplasms of different morphology, etiology, and pathogenesis.

Aim

In this review, the etiology, pathogenesis, diagnostic criteria, classification, and molecular alterations of intra- and extrahepatic cholangiocarcinomas are summarized.

Material and Methods

This review is based on the currently available literature and personal knowledge.

Results and Discussion

Cholangiocarcinomas are morphologically and molecularly diverse neoplasms that can develop ubiquitously in the biliary tract. Since there is a significant histological and immunohistochemical overlap with frequent liver metastases, a definite diagnosis can often only be rendered in the clinical context. Cholangiocarcinomas are subclassified into intrahepatic (iCCA), perihilar (pCCA), and distal (dCCA) tumors according to macro-anatomical criteria. Recent studies show that there are distinctive molecular differences in particular between iCCA and extrahepatic CCA (eCCA, including pCCA and dCCA). In addition, morphologically well-characterized precursor lesions have been identified, which in various frequencies can be assigned not only to the anatomical classification, but also to certain etiologies. An exact classification is therefore essential, especially with regard to the development of innovative, targeted therapeutic approaches.

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Authors and Affiliations

  1. Pathologisches Institut, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 224, 69120, Heidelberg, Deutschland

    Benjamin Goeppert

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Correspondence to Benjamin Goeppert.

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B. Goeppert gibt an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden vom Autor keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Schwerpunktherausgeber

H. Baba, Essen

T. Longerich, Heidelberg

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Goeppert, B. Cholangiokarzinome – Übersicht zur aktuellen anatomischen, histomorphologischen und morphomolekularen Klassifikation. Pathologe 41, 488–494 (2020). https://doi.org/10.1007/s00292-020-00808-6

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