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Neuroendokrine Neoplasien

Neuroendocrine neoplasms

  • Leitthema
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Der Onkologe Aims and scope

Zusammenfassung

Hintergrund

Neuroendokrine Neoplasien (NEN) stellen eine heterogene Erkrankung mit unterschiedlicher klinischer Präsentation und Prognose dar. Die Tumoren befinden sich häufig im Verdauungstrakt und werden dort als gastroenteropankreatische (GEP‑)NEN bezeichnet. NEN machen etwa 1 % aller Malignome aus und zählen damit zu den seltenen Krebserkrankungen des Menschen.

Ziel und Methoden

Die vorliegende Arbeit gibt anhand der Literatur eine Übersicht über die Merkmale von NEN.

Ergebnisse

GEP-NEN zeigen abhängig von Differenzierung, anatomischer Lage, Größe, Proliferationsindex Ki-67 und Hormonfreisetzung unterschiedlichste Symptome und Verläufe. Die funktionell aktiven GEP-NEN stellen aufgrund ihrer Hormonexzesssyndrome besondere Anforderungen an Diagnostik und Therapie. Zu den antiproliferativen Therapieoptionen gehören Somatostatinanaloga, molekular-zielgerichtete Therapien (Sunitinib, Everolimus), Peptid-Radiorezeptor-Therapie und die systemische Chemotherapie. Die Zahl der Phase-III-Studien ist limitiert, komparative Studien fehlen für die meisten Situationen. Eine etablierte Therapiesequenz ist daher nur selten vorhanden, die Maßnahmen werden individualisiert eingesetzt.

Schlussfolgerung

Therapieziel bei differenzierten neuroendokrinen Tumoren ist eine Wachstumsstabilisierung. Bei undifferenzierten neuroendokrinen Karzinomen ist die objektive Ansprechrate auf eine systemische Chemotherapie höher, jedoch statistisch von kurzer Dauer.

Abstract

Background

Neuroendocrine neoplasias (NEN) are a heterogeneous group of diseases with distinct clinical presentations and prognoses. They frequently occur in the digestive tract, where they are referred to as gastroenteropancreatic (GEP) NEN. NEN account for about 1% of all malignancies and are therefore among the rare human cancers.

Objective and methods

Based on the literature, the current article provides an overview of the characteristics of NEN.

Results

Depending on their differentiation, anatomic location, size, Ki-67 proliferation index, and hormone release, GEP-NEN exhibit a wide variety of symptoms. Due to hormone excess syndromes, functionally active GEP-NEN require dedicated diagnostic strategies and treatment. Medical therapies include somatostatin analogues, targeted therapies (sunitinib, everolimus), peptide receptor radionucleotide therapy, and systemic chemotherapy. The number of phase III studies is limited, comparative trials are lacking in most cases. Therefore, a clear treatment algorithm is rarely available and treatments are applied in a sequential and individualized manner.

Conclusion

The therapeutic goal in differentiated neuroendocrine tumors is disease stabilization. Chemotherapy may induce partial tumor remissions in undifferentiated neuroendocrine carcinomas, but these are usually short lasting.

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Authors and Affiliations

  1. Klinik für Endokrinologie, Diabetologie und Stoffwechsel, Endokrines Tumorzentrum am WTZ mit ENETS Center of Excellence, Europäisches Referenzzentrum für seltene endokrine Tumoren (ENDO-ERN, EURACAN), Universitätsmedizin Essen, Universität Duisburg-Essen, Essen, Deutschland

    Harald Lahner & Dagmar Führer

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  1. Harald Lahner
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  2. Dagmar Führer
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Correspondence to Harald Lahner.

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Interessenkonflikt

H. Lahner: Beratungs- und Referententätigkeit: Ipsen, Novartis, Pfizer. D. Führer gibt an, dass kein Interessenkonflikt besteht.

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

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Lahner, H., Führer, D. Neuroendokrine Neoplasien. Onkologe 26, 261–269 (2020). https://doi.org/10.1007/s00761-019-00705-5

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