Zusammenfassung
Anhand zweier Beobachtungen und eines Literaturüberblickes werden Pathologie und Klinik der seltenen neurogenen Sarkome im Halsbereich bei Patienten mit Neurofibromatose geschildert. Diese Tumoren zeigen histologisch ein vielfältiges Bild: neben spindel-zelligen Sarkomen finden sich pleomorphe Geschwülste, die u. a. einem Rhabdomyo-, Lipo-, Chondro-, Angio- oder osteogenen Sarkom ähneln können. Ohne klinische Angaben ist die histologische Diagnose eines neurogenen Sarkoms nicht immer sicher zu stellen. Bis heute steht die chirurgische Behandlung im Vordergrund. Der Wert einer Chemotherapie und der Bestrahlung ist umstritten. Die Behandlungsergebnisse bei diesen Tumoren sind unbefriedigend: unter 29 Berichten aus der Literatur fanden sich lediglich zwei mit Heilungen über die 5-Jahresgrenze hinaus.
Summary
Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than 5 years.
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Read in part at the 63rd Annual Meeting of the Nordwestdeutsche Vereinigung der Hals-Nasen-Ohrenärzte, Timmendorfer Strand, October 1980
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Martin, G., Kleinsasser, O. Neurogenic sarcomas of the neck in neurofibromatosis. Arch Otorhinolaryngol 232, 273–283 (1981). https://doi.org/10.1007/BF00457453
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DOI: https://doi.org/10.1007/BF00457453
Schlüsselwörter
- Neurogenes Sarkom
- Nervenscheidentumoren
- Neurofibromatosis
- Kopf- und Halstumoren
- Tumoren im Kindesalter
- Histopathologie