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Neuroendokrine Neoplasien

Zwei Familien mit sehr unterschiedlichen Charakteristika vereint in einer Klassifikation

Neuroendocrine neoplasms

Two families with distinct features unified in one classification (German version)

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  • Dieser Beitrag wurde in der Zeitschrift Der Pathologe 3/2019 · 40:211–219 https://doi.org/10.1007/s00292-019-0594-3 erstveröffentlicht. Aktualisierte Zweitpublikation mit freundlicher Genehmigung des Autors.

Zusammenfassung

Alle neuroendokrinen Neoplasien (NEN) sind durch die Expression von Synaptophysin und Chromogranin A (oder B) charakterisiert, dennoch sind sie keine homogene Tumorgruppe. Paradigmatisch für diese Tumoren sind die NEN des gastroenteropankreatischen (GEP) Systems. Hier stehen sich 2 NEN-Familien gegenüber: Überwiegend gut differenzierte und gering proliferative NEN, auch neuroendokrine Tumoren (NET) genannt, sowie schlecht differenzierte und hoch proliferative NEN, die als neuroendokrine Karzinome (NEC) bezeichnet werden. GEP-NET werden weiterhin anhand ihrer proliferativen Aktivität in G1, G2 und G3 unterteilt. NEC sind definitionsgemäß G3-Karzinome. Die morphologische Dichotomie der NEN wird begleitet von Unterschieden in der Epidemiologie, Genetik, Klinik und Prognose und hat möglicherweise ihre Ursache in einer Abstammung aus unterschiedlichen Progenitorzellen. Genetisch zeichnen sich die NEC durch TP53- und RB1-Alterationen aus, die den NET fehlen und bei der Unterscheidung von NET G3 zu NEC hilfreich sind. Der Vergleich der GEP-NEN-WHO-Klassifikation mit NEN-Klassifikationen anderer Organsysteme offenbart Unterschiede in der Terminologie und den Kriterien der Kategorisierung. Außerdem fehlt ein Gradingsystem. Gemeinsam ist allen NEN-Klassifizierungen jedoch, dass sie zwischen 2 Tumorfamilien mit unterschiedlicher Differenzierung und Prognose unterscheiden. Dies erlaubt, den NEN einen einheitlichen Klassifikationsrahmen zu geben.

Abstract

All neuroendocrine neoplasms (NENs) are characterized by the expression of synaptophysin and chromogranin A (or B). Yet, they are not a homogeneous group of tumors. Paradigmatic for these tumors are the NENs of the gastroenteropancreatic (GEP) system. Two NEN families can be distinguished: predominantly well differentiated and low-proliferative NENs, called neuroendocrine tumors (NET), and poorly differentiated and high-proliferative NENs, called neuroendocrine carcinomas (NECs). Based on their proliferative activity, GEP NETs are further classified into G1, G2, and G3 tumors. NECs are per definition G3 carcinomas. The morphological NEN dichotomy is supported by differences in epidemiology, genetics, clinics, and prognosis, and potentially has its cause originating from different progenitor cells. Genetically, NECs are distinguished by TP53 and RB1 alterations, which are lacking in NETs and are helpful in the distinction of NETs from NECs. Comparison of the GEP NEN WHO classification with extragastroenteropancreatic NEN classifications commonly reveal differences in terminology and categorization. In addition, they lack a grading system. However, common to all NEN classifications is the recognition of two tumor families differing in histological differentiation and prognosis. This allows the construction of a uniform classification frame for all NENs.

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Correspondence to Prof. Dr. Günter Klöppel.

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Klöppel, G. Neuroendokrine Neoplasien. Wien klin Mag (2020). https://doi.org/10.1007/s00740-019-00325-0

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Schlüsselwörter

  • Chromogranine
  • Gastrointestinaltrakt
  • Neuroendokrine Karzinome
  • Pankreastumoren
  • Zelldifferenzierung

Keywords

  • Chromogranins
  • Gastrointestinal tract
  • Neuroendocrine carcinomas
  • Pancreatic neoplasms
  • Cell differentiation