Summary
Malignant peripheral nerve sheath tumors are rare and most often appear in the clinical setting of von Recklinghausen's neruofibromatosis. On rare occasions they are seen in patients lacking any of the overt stigmata of this systemic disease. We report on a case of a peripheral nerve sheath sarcoma (PNSS) on the scalp and the back of the neck of a 13-year-old female arising in a solitary benign neurofibroma with no evidence of systemic neurofibromatosis. The tumor was examined by ultrastructural and immunohistochemical techniques as well as by routine histopathology. It was defined as a peripheral nerve sheath sarcoma, fibrosarcomatous type, of poorly differentiated perineurial cell origin. The possible significance of histogenetic subclassification in terms of surgical management and the oncological approach as well as the prognosis is discussed.
Similar content being viewed by others
References
D'Agostini AN, Soule EH, Miller RH (1983) Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (von Recklinghausen's disease). Cancer 16:1003
Donaldson SS (1985) The value of adjuvant chemotherapy in the management of sarcomas in children. Cancer 55:2184
Ducatman BS, Scheithauser BW, Piepgras DG, Reiman MM, Ilstrup DN (1986) Malignant peripheral nerve sheath tumors. Cancer 57:2006
Favara BE, Galliani CA, Wakely PE Jr (1986) Advances in the case of the child with cancer. The importance of histologic subclassification of tumors. Cancer 58:426
Leroy JP, Volant A, Mazabrand A (1984) Les sarcomas d'origine nerveuse. Arch Anat Cytol Pathol 32:333
Miser JS, Pizzo PA (1985) Soft tissue sarcomas in childhood. Pediatr Clin North Am 32:779
Mogollon R, Penneys N, Albores-Saavedra J, Nadji M (1984) Malignant schwannoma presenting as a skin mass. Confirmation by the demonstration of myelin basic protein within tumor cells. Cancer 53:1190
Morgan KG, Gray C (1985) Malignant epithelioid schwannoma of superficial soft tissue. A case report with immunohistology and electronmicroscopy. Histopathology 9:765
Naujoka JH, Wuensch PH, Ratzka M, Uffenorde J (1981) Das neurogene Sarkom in Kopf-Halsbereich mit Kontakt zur Schädelbasis. Arch Otorhinolaryngol 233:271
Storm FK, Eilber FR, Mirra J, Morton DL (1980) Neurofibrosarcoma. Cancer 45:126
Tsukahara Y, Sato M, Kato J et al. (1985) Case report of malignant primary nerve tumor arising in the female pelvis. Acta Obstet Gynecol Jpn 37:1915
Woodruff JM, Marshall ML, Godwin TA, Funkhouser JW, Thompson NJ, Erlandson RA. Plexiform (multinodular) schwannoma. A tumor simulating the plexiform neurofibroma. Am J Surg Pathol 7: 691
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Hauben, D.J., Sarig, A., Goldstein, J. et al. A rare case of a solitary peripheral nerve sheath sarcoma of the scalp. Eur J Plast Surg 13, 36–39 (1990). https://doi.org/10.1007/BF00184403
Issue Date:
DOI: https://doi.org/10.1007/BF00184403