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Das neurogene Sarkom im Kopf-Halsbereich mit Kontakt zur Schädelbasis

Neurogenic sarcoma of the head and neck with contact to the skull base

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Summary

The neurogenic sarcoma is a neoplasm found seldom. It descends from the sheaths of peripheric nerves. If this tumor develops from a solitary or multiple neurofibroma, it might also be called a neurofibrosarcoma. All other synonyms, especially that of the malignant schwannoma, should better be abandoned for histogenetic reasons. Today the classification of all tumors of the peripheral nervous system is based on a suggestion of the WHO from 1969. The tabular summary of the literature shows that only a little more than a hundred cases of neurogenic sarcoma of the head and neck were reported during the last 50 years.

Differential diagnosis of this malignant tumor includes nearly all other malignant neoplasms of soft tissue, sometimes even anaplastic carcinoma.

Histopathology, including electron microscopy, is described in general and also in detail with regard to three cases.

Three forms of micro-morphological variants are dealt with, too. For diagnosis histological examination will be absolutely successful only in cases of neurofibromatosis or in cases where the nerve from which the tumor originates may be identified. Otherwise, conventional and computerized X-ray tomography may be helpful. Finally, some therapeutic and prognostic principles are discussed.

Zusammenfassung

Das neurogene Sarkom ist ein insgesamt seltenes malignes Neoplasma, das sich von den Hüllen des peripheren Nerven ableitet. Entwickelt sich diese Geschwulst aus einem solitären oder multiplen Neurofibrom, so wäre nomenklatorisch auch die Bezeichnung Neurofibrosarkom gerechtfertigt, während alle anderen Synonyma für diese Geschwulst, insbesondere das maligne Schwannom, aus histogenetischen Gründen verlassen werden sollten. Für die Klassifizierung aller Tumoren des peripheren Nervensystems ist heute ein von der WHO (1969) akzeptierter Vorschlag zugrunde zu legen. Ein tabellarischer Literaturüberblick zeigt, daß in den letzten 50 Jahren nur über wenig mehr als 100 Fälle von neurogenen Sarkomen im Kopf-Halsbereich berichtet wurde.

Die Differentialdiagnose dieses malignen Tumors umfaßt nahezu alle anderen malignen Weichgewebsgeschwülste und manchmal sogar ein anaplastisches Karzinom.

Die Histopathologie, einschließlich der Elektronenmikroskopie, wird allgemein und an drei eingehenden Falldemonstrationen aufgezeigt, wobei auch den drei mikromorphologischen Varianten Rechnung getragen wird. Neben der feingeweblichen Untersuchung, die nur beim zusätzlichen Vorliegen einer Neurofibromatose oder bei der Identifizierung des Ursprungsnerven hundertprozentig gesichert werden kann, sind konventionelle und computertomografische Röntgentechniken zur Diagnosestellung hilfreich. Zum Schluß werden die Grundsätze der Therapie sowie die Prognose diskutiert.

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Authors and Affiliations

  1. Universitätsklinik und Poliklinik für Hals-, Nasen- und Ohrenkranke im Kopfklinikum Würzburg, D-8700, Würzburg, Bundesrepublik Deutschland

    J. H. Naujoks & J. Uffenorde

  2. Pathologisches Institut der Universität Würzburg, D-8700, Würzburg

    P. H. Wünsch

  3. Abteilung für Neuroradiologie der Universität Würzburg, D-8700, Würzburg

    M. Ratzka

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  1. J. H. Naujoks
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  2. P. H. Wünsch
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  3. M. Ratzka
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  4. J. Uffenorde
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Da es sich bei dieser Arbeit um eine wichtige Zusammenstellung handelt, erfolgt die Publikation ausnahmsweise ungekürzt

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Naujoks, J.H., Wünsch, P.H., Ratzka, M. et al. Das neurogene Sarkom im Kopf-Halsbereich mit Kontakt zur Schädelbasis. Arch Otorhinolaryngol 233, 271–300 (1981). https://doi.org/10.1007/BF00454391

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  • DOI: https://doi.org/10.1007/BF00454391

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