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Neuroendokrine Neoplasien

Neuroendocrine neoplasms

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Zusammenfassung

Der Gastrointestinaltrakt ist zusammen mit der Lunge der häufigste Ort für das Auftreten neuroendokriner Neoplasien (NEN). Zu den NEN gehören 2 Hauptgruppen von Entitäten: die gut differenzierten neuroendokrinen Tumoren (NET), die zwar alle potenziell maligne sind, aber in der Regel eine gute Prognose haben, und die schlecht differenzierten und klinisch aggressiven neuroendokrinen Karzinome (NEC). Eine dritte Gruppe bilden Neoplasien, die sowohl eine neuroendokrine als auch eine nichtneuroendokrine Differenzierung aufweisen, sog. gemischte neuroendokrine und nichtneuroendokrine Neoplasien (MiNEN). Die relative Häufigkeit von NET und NEC unterscheidet sich in den unterschiedlichen anatomischen Lokalisationen entlang der Speiseröhre, des Magens und des Dünn- und Dickdarms, was sich auf das Spektrum der klinischen Erscheinungsformen auswirkt. Die Diagnose von NEN und ihre Klassifizierung beruhen auf gut etablierten immunhistochemischen Markern. Die molekulare Pathogenese von NEN, die in einigen Fällen ihrem biologischen Verhalten zugrunde liegt, wird derzeit aufgeklärt; molekulare Biomarker für personalisierte Behandlungsansätze fehlen jedoch weitgehend.

Abstract

The gastrointestinal tract represents, together with the lung, the most common site for the occurrence of neuroendocrine neoplasms (NEN). NEN include two main groups of entities: the well-differentiated neuroendocrine tumors (NET), which, although all being potentially malignant, usually bear a good prognosis and the poorly differentiated and clinically aggressive neuroendocrine carcinomas (NEC). A third group is represented by neoplasms displaying a neuroendocrine and a nonneuroendocrine differentiation, so-called mixed neuroendocrine and nonneuroendocrine neoplasms (MiNEN). The relative frequency of NET and NEC differs depending on the anatomical location along the esophagus, stomach and intestine, which also affects the spectrum of clinical presentations. The diagnosis of NEN and their classification relies on well-established immunohistochemical markers. The molecular pathogenesis of NEN, which in some cases underlies their biological behavior, is currently being clarified; however, molecular biomarkers for personalized treatment approaches are mostly missing.

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  1. Institut für Pathologie, Heinrich-Heine-Universität und Universitätsklinikum Düsseldorf, Moorenstr. 5, 40225, Düsseldorf, Deutschland

    Irene Esposito, Lena Häberle & Aslihan Yavas

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  1. Irene Esposito
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  2. Lena Häberle
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  3. Aslihan Yavas
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Correspondence to Irene Esposito.

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I. Esposito, L. Häberle und A. Yavas geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Irene Esposito, Düsseldorf

Andrea Tannapfel, Bochum

Eva Wardelmann, Münster

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Der Verlag bleibt in Hinblick auf geografische Zuordnungen und Gebietsbezeichnungen in veröffentlichten Karten und Institutsadressen neutral.

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Esposito, I., Häberle, L. & Yavas, A. Neuroendokrine Neoplasien. Gastroenterologie (2024). https://doi.org/10.1007/s11377-024-00784-w

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