Histo- und Molekularpathologie bei gastroenteropankreatischen neuroendokrinen Neoplasien

Die pathologische Differenzierung der neuroendokrinen Neoplasien (NEN) leistet einen wichtigen Beitrag in der Primärdiagnostik und kann in der metastasierten Situation bei unklarem Primarius den entscheidenden Hinweis auf den Primärtumor liefern. Histologische und molekularpathologische Merkmale der NEN können in das klinisch-therapeutische Management einbezogen werden und werden im Folgenden bei NEN des gastroenteropankreatischen Systems vorgestellt.

Abstract

Neuroendocrine neoplasms are classified according to the WHO classification based on morphological criteria into neuroendocrine tumors, neuroendocrine carcinomas, and mixed neuroendocrine-non-neuroendocrine neoplasms. Neuroendocrine tumors are well differentiated neoplasms and show characteristic site-specific histological and molecular features, which is important for their clinical management. In cases dealing with metastasis, pathology often can help to identify the primary tumors using a small immunohistochemical marker panel. Neuroendocrine carcinomas are poorly differentiated neoplasms. They are subdivided into neuroendocrine carcinomas of small cell and large cell type. The molecular profile of neuroendocrine carcinomas and mixed neuroendocrine-non-neuroendocrine neoplasms shows a close relationship to conventional adenocarcinomas with site-specific features. Molecular analysis of neuroendocrine carcinomas and neuroendocrine-non-neuroendocrine neoplasms are not yet fully integrated in daily diagnostics and are mainly performed in the context of precision oncology.

Kernaussagen

• Die WHO-Klassifikation teilt die neuroendokrinen Neoplasien anhand morphologischer Kriterien in neuroendokrine Tumoren, neuroendokrine Karzinome und gemischte neuroendokrine, nicht neuroendokrine Neoplasien ein.

• Neuroendokrine Tumoren sind gut differenziert und zeigen lokalisationsspezifisch charakteristische histologische und molekulare Merkmale, die essenziell für das klinisch-therapeutische Management sind.

• In der Diagnostik von Metastasen bei unklarem Primarius kann die pathologisch-anatomische Diagnostik mit einem kleinen immunhistochemischen Markerpanel den entscheidenden Hinweis für die Lokalisation des Primarius liefern.

• Neuroendokrine Karzinome sind schlecht differenziert und werden weiter in kleinzellige und großzellige Typen unterteilt.

• Das molekulare Profil der neuroendokrinen Karzinome und der gemischten neuroendokrinen, nicht neuroendokrinen Neoplasien zeigt organspezifische Merkmale und orientiert sich an dem genetischen Profil der konventionellen Adenokarzinome.

• Die molekularen Analysen sind bei den neuroendokrinen Karzinomen und den gemischten neuroendokrinen, nicht neuroendokrinen Neoplasien noch nicht vollständig in den diagnostischen Alltag integriert und werden zurzeit vor allem im Rahmen von molekularen Tumorboards durchgeführt.

Schlüsselwörter

neuroendokriner Tumor - neuroendokriner Tumor - Neoplasie - Ki67 - Molekularpathologie

Keywords

neuroendocrine tumor - neuroendocrine carcinoma - neoplasm - Ki67 - molecular pathology

Publication History

Article published online:
16 July 2024

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