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Multifokale Manifestation einer Rosai-Dorfman-Erkrankung

Seltene, rein extranodale Sinushistiozytose im Erwachsenenalter

Multifocal manifestation of Rosai–Dorfman disease

A rare case of purely extranodal sinus histiocytosis in the elderly

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Zusammenfassung

Die Rosai-Dorfman-Erkrankung zählt zu den kindlichen Histiozytosen und wurde initial als Sinushistiozytose mit massiver Lymphadenopathie (SHML) beschrieben. In ihrer rein extranodalen Verlaufsform betrifft sie vorzugsweise Strukturen von Kopf und Hals. Anhand einer atypischen Erstmanifestation im hohen Lebensalter mit multifokaler, rein extranodaler Organbeteiligung wird dieses seltene Krankheitsbild hinsichtlich seiner spezifischen Eigenschaften beleuchtet. Dabei werden insbesondere die (differenzial)diagnostischen Schwierigkeiten und die individuell angepassten Therapieoptionen in ihrer Wertigkeit dargelegt.

Abstract

Rosai–Dorfman disease belongs to the group of childhood histiocytoses and was initially described as sinus histiocytosis with massive lymphadenopathy. Its rare purely extranodal manifestation is primarily found in the head and neck region. An atypical primary manifestation in an elderly patient with multifocal extranodal disease is described, and this pathological entity is reviewed. Specific difficulties concerning differential diagnostic aspects as well as individually appropriate treatment strategies are discussed.

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Danksagung

Für die unbürokratische Bereitstellung der in diesem Artikel verwandten Computertomographien möchten wir allen beteiligten Mitarbeitern des Instituts für Neuroradiologie der Julius-Maximilians-Universität Würzburg (Direktor: Prof. Dr. med. Solymosi) ausdrücklich danken.

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Authors and Affiliations

  1. Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf-, Hals- und plastische Gesichtschirurgie, Klinikum Darmstadt, Heidelberger Landstraße 379, 64297, Darmstadt, Deutschland

    I. Ott & G. Baier

  2. Klinik für Hals-Nasen-Ohren-Krankheiten, Kopf-, Hals- und plastische Gesichtschirurgie, Kommunikationsstörungen, Städtisches Klinikum, Fulda, Deutschland

    M. Weber & K. Schwager

  3. Klinik und Poliklinik für Hals-, Nasen- und Ohrenkranke, Kopf- und Halschirurgie, Bayerische Julius-Maximilians-Universität, Würzburg, Deutschland

    M. Weber & R. Hagen

  4. Institut für Pathologie, Bayerische Julius-Maximilians-Universität, Würzburg, Deutschland

    S. Gattenlöhner

Authors
  1. I. Ott
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  2. M. Weber
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  3. S. Gattenlöhner
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  4. K. Schwager
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  5. R. Hagen
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  6. G. Baier
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Corresponding author

Correspondence to I. Ott.

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Ott, I., Weber, M., Gattenlöhner, S. et al. Multifokale Manifestation einer Rosai-Dorfman-Erkrankung. HNO 58, 263–267 (2010). https://doi.org/10.1007/s00106-008-1861-4

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  • DOI: https://doi.org/10.1007/s00106-008-1861-4

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