Abstract
Malignant schwannomas are rare neoplasms that are seldom found in the head and neck. Few cases have been reported involving paranasal sinuses and none of them was of the “epithelioid” type, In this report, an unusual case of epithelioid malignant schwannoma involving the maxillary sinus, nasal cavity and orbit is presented. The patient was a 27-year-old male with a history of headache, nasal obstruction and epistaxis. Histologically, the tumour had a biphasic pattern with spindle and epithelioid elements which led to a differential diagnosis with malignant melanoma. It had also to be distinguished from other neoplasms, such as squamous cell carcinoma and olfactory neuroblastoma because of it location. Immunohistochemical positivity for S-100 protein, glial fibrillary acidic protein and vimentin together with negativity for HMB-45 and cytokeratins, as well as mesaxon formation detected with electron microscopy were conclusive in the diagnosis. The patient was treated with surgical excision and radiotherapy but local recurrence and metastases occurred, and he died within 1 year after initial diagnosis.
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Abell MR, Hart WR, Olson JR (1970) Tumors of the peripheral nervous system. Hum Pathol 1:503–551
Alvira MM, Mandybur TI, Menefee MG (1976) Light microscopic and ultrastructural observations of a metastasizing malignant epithelioid schwannoma. Cancer 38:1977–1982
Bailet JW, Abemayor E, Andrews JC, Rowland JP, Fu Y-S, Dawson DE (1991) Malignant nerve sheath tumours of the head and neck: a combined experience from two university hospitals. Laryngoscope 101:1044–1048
Cardesa A, Alos LI, Palacin A, Bombi JA, Traserra J (1991) Relative frequency and differential diagnoses of sinonasal malignant melanoma (SNMM) (abstract). Lab Invest 64:63A
Chu FA, Shmookler BM (1988) Malignant epithelioid schwannoma: a light microscopic and immunohistochemical study. J Surg Oncol 39:68–72
Conley J, Janecka IP (1975) Neurilemmoma of head and neck. Trans Am Acad Ophthalmol Otolaryngol 80:459–464
D'Agostino AN, Soule EH, Miller RA (1963) Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (von Recklinghausen's disease). Cancer 16:1015–1027
DiCarlo EF, Woodruff JM, Bansal M, Erlandson RA (1986) The purely epithelioid malignant peripheral nerve sheath tumour. Am J Surg Pathol 10:478–490
Erlandson RA, Woodruff JM (1982) Peripheral nerve sheath tumours: an electron microscopic study of 43 cases. Cancer 49:273–287
Goepfert H, Lindberg RD, Sinkovics JG, Ayala AG (1977) Soft-tissue sarcoma of the head and neck after puberty. Arch Otolaryngol 103:365–368
Grätz KW, Makek M, Sailer HF (1991) Malignant melanotic schwannoma of the oral cavity. Int J Oral Maxillofac Surg 20:236–238
Greager JA, Reichard KW, Campana JP, DasGupta TK (1992) Malignant schwannoma of the head and neck Am J Surg 63:440–442
Honma K, Watanabe H, Ohnishi Y, Tachikawa S, Tachikawa K (1989) Epitheliod malignant schwannoma. A case report. Acta Pathol Jpn 39:195–202
Kragh LV, Soule EH, Masson JK (1960) Benign and malignant neurilemmomas of the head and neck. Surg Gynecol Obstet 111:211–213
Laskin WB, Weiss S, Bratthauer GL (1991) Epithelioid variant of malignant peripheral nerve sheath tumour (malignant epithelioid schwannoma). Am J Surg Pathol 15:1136–1145
Lodding P, Kindblom L-G, Angervall L (1986) Epithelioid malignant schwannoma. A study of 14 cases. Virchows Arch [A] 409:433–451
Millard F, Busser F (1952) Schwannoma de maxillaire superieur d'evolution maligne. Ann Otolaryngol 69:706–708
Morgan KG, Gray C (1985) Malignant epithelioid shwannoma of superficial soft-tissue? A case report with immunohistology and electron microscopy. Histopathology 9:765–775
Perzin KH, Panyu H, Wechter S (1982) Nonepithelial tumours of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. XII: Schwann cell tumours (neurilemoma, neurofibroma, malignant schwannoma). Cancer 50:2193–2202
Robitaille Y, Seemayer TA, EI Deiry A (1975) Peripheral nerve tumours involving paranasal sinuses: a case report and review of the literature. Cancer 35:1254–1258
White HR (1971) Survival in malignant schwannoma. Cancer 27:720–729
Younis RT, Gross CW, Lazar RH (1991) Schwannomas of the paranasal sinuses. Arch Otolaryngol Head Neck Surg 117:677–680
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Fernández, P.L., Cardesa, A., Bombí, J.A. et al. Malignant sinonasal epithelioid schwannoma. Vichows Archiv A Pathol Anat 423, 401–405 (1993). https://doi.org/10.1007/BF01607154
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DOI: https://doi.org/10.1007/BF01607154