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Neuroendokrine Neoplasien der Kopf-Hals-Region

Neuroendocrine neoplasms of the head and neck

  • Schwerpunkt: Kopf-Hals-Tumoren
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Zusammenfassung

Allen neuroendokrinen Neoplasien (NEN), gleich welcher Lokalisation und Differenzierung, ist die Expression von Synaptophysin und Chromogranin A gemeinsam. In der Kopf-Hals-Region sind NEN entweder epithelialer oder neuraler/neuroektodermaler Herkunft, wobei die beiden genannten Gruppen sich durch die Expression von Zytokeratinen unterscheiden. NEN epithelialen Ursprungs sind die typischen und atypischen Karzinoide (von der WHO auch als „low-“ und „intermediate-grade“ neuroendokrine Karzinome bezeichnet), die schlecht differenzierten „high-grade“ neuroendokrinen Karzinome vom klein- und großzelligen Typ und die gemischten neuroendokrinen-nichtneuroendokrinen Neoplasien. Die neural/neuroektodermalen NEN umfassen die Olfaktoriusneuroblastome und Paragangliome. Olfaktoriusneuroblastome werden nach Hyams in 4 Differenzierungsgrade stratifiziert. Paragangliome der Kopf-Hals-Region treten gehäuft multipel und familiär auf. Die meisten NEN der Kopf-Hals-Region sind im oberen Respirationstrakt anzutreffen. Die Diagnostik folgt den generellen Richtlinien für NEN, wobei immunhistochemische Untersuchungen im Mittelpunkt stehen. Molekularpathologische Untersuchungen bilden die Ausnahme.

Abstract

Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms. The neural-neuroectodermal-type NENs comprise olfactory neuroblastoma and paraganglioma, each of them with distinct clinicopathological characteristics. Olfactory neuroblastomas show a spectrum of histologic differentiation and are prognostically classified by Hyams grading. Paragangliomas often occur multiple and show a familial background. Most head and neck NENs occur in the upper respiratory system. Their diagnosis follows the general guidelines for NENs, focusing on immunohistochemical profiling. Molecular examinations are so far only required in individual cases.

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Authors and Affiliations

  1. Institut für Pathologie, Technische Universität München, Trogerstr. 18, 81675, München, Deutschland

    B. Konukiewitz, W. Weichert & G. Klöppel

  2. Institut für Pathologie, Universitätsklinikum Erlangen, Erlangen, Deutschland

    A. Agaimy

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  1. B. Konukiewitz
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  4. G. Klöppel
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Correspondence to B. Konukiewitz.

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Interessenkonflikt

B. Konukiewitz, A. Agaimy, W. Weichert und G. Klöppel geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Schwerpunktherausgeber

A. Hartmann, Erlangen

A. Agaimy, Erlangen

S. Ihrler, München

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Konukiewitz, B., Agaimy, A., Weichert, W. et al. Neuroendokrine Neoplasien der Kopf-Hals-Region. Pathologe 39, 27–34 (2018). https://doi.org/10.1007/s00292-017-0404-8

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  • DOI: https://doi.org/10.1007/s00292-017-0404-8

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