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Der Pathologe

, Volume 38, Issue 6, pp 521–528 | Cite as

Tumoren des Innenohrs und angrenzender Strukturen

  • J. Schittenhelm
Schwerpunkt: Tumoren der Sinnesorgane

Zusammenfassung

Tumoren des Innenohrs und der angrenzenden Strukturen manifestieren sich aufgrund der Druckwirkung auf die durchziehenden Hirnnerven häufig durch Innenohrschwerhörigkeit, Tinnitus oder vestibulären Schwindel. Über 90 % der Raumforderungen im inneren Gehörgang mit oder ohne Ausdehnung im Kleinhirnbrückenwinkel werden histologisch als Vestibularisschwannome identifiziert. Zu den weniger häufigen tumorösen Läsionen zählen die ektop gelegenen Felsenbeinmeningeome, Paragangliome des Glomus tympanicum oder jugulare sowie die speziell vom Recessus vestibularis ausgehenden Tumoren des endolymphatischen Sackes (ELST). Auch wenn die meisten Entitäten sporadisch sind, muss an die Möglichkeit hereditärer Tumorerkrankungen wie die Neurofibromatose Typ 2 bei bilateralen Schwannomen und an die von-Hippel-Lindau-Erkrankung bei Kombination von ELST mit anderen Tumoren gedacht werden. Das weitere therapeutische Vorgehen wird im Wesentlichen durch die (neuro-)pathologische Diagnose und die Gradierung der Tumoren bestimmt.

Schlüsselwörter

Hirnnerven Neuropathologie ELST NF2 VHL 

Tumors of the inner ear and adjacent structures

Abstract

Tumors of the inner ear and adjacent structures often present with hearing loss, tinnitus and vertigo due to compression of the traversing cranial nerves. More than 90% of the tumors of the inner ear with or without expansion into the cerebellopontine angle are histologically diagnosed as vestibular schwannomas. Less common tumorous lesions include ectopic meningiomas located in the petrous bone, glomus tympanicum paragangliomas or endolymphatic sac tumors (ELST) originating in the vestibular recess. Most tumors are sporadic, but hereditary disorders have to be considered. Bilateral vestibular schwannomas are indicative of neurofibromatosis type 2 and ELST in conjunction with other abdominal tumors indicates von Hippel-Lindau disease. The neuropathological diagnostics and grading guides the subsequent therapy of these mostly benign lesions.

Keywords

Cranial nerves Neuropathology ELST NF2 VHL 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

J. Schittenhelm gibt an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine vom Autor durchgeführten Studien an Menschen oder Tieren.

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Copyright information

© Springer Medizin Verlag GmbH 2017

Authors and Affiliations

  1. 1.Abteilung Neuropathologie, Department für Pathologie und Neuropathologie und Zentrum für Neuroonkologie, Comprehensive Cancer Center, Universitätsklinikum TübingenEberhard-Karls-Universität TübingenTübingenDeutschland

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