A Rare Case Report of Malignant Peripheral Nerve Sheath Tumor in the Infratemporal Fossa.

Molham Alahmad, Lubna Kharita, Arige Alassaf
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Abstract

Introduction: Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors that develop from peripheral nerve sheath cells and they account for approximately 5% to 10% of all soft-tissue sarcomas. MPNSTs in the head and neck region represent approximately 2% to 6% of all head and neck sarcomas and account for 12% to 19% of all MPNSTs, and the infratemporal fossa is a rare site for MPNSTs. MPNSTs originating from the trigeminal nerve are extremely rare. Case presentation: A 19-year-old female presented with a 6-month history of left-sided facial pain and paresthesia on the same side. On examination, there was left-sided facial paresthesia at the third trigeminal nerve (V3) areas; computed tomography scanning and magnetic resonance imaging showed an infratemporal lesion and surgical resection was done. Histological examination and the immunostaining finding showed high-grade MPNST. Conclusion: MPNSTs in the head and neck region may manifest with nonspecific symptoms. The diagnosis often requires a combination of clinical, pathological, and immunohistochemistry studies. Treatment involves total surgical resection with adjuvant radiotherapy.

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颞下窝恶性周围神经鞘瘤罕见病例报告
简介恶性周围神经鞘瘤(MPNST)是由周围神经鞘细胞发展而来的罕见肿瘤,约占所有软组织肉瘤的5%至10%。头颈部的 MPNST 约占所有头颈部肉瘤的 2% 至 6%,占所有 MPNST 的 12% 至 19%,而颞下窝是 MPNST 的罕见部位。源自三叉神经的多发性肉芽肿极为罕见。病例介绍:一名 19 岁女性因左侧面部疼痛和同侧麻痹 6 个月前来就诊。经检查,左侧面部第三三叉神经(V3)区域有麻痹感;计算机断层扫描和磁共振成像显示颞下病变,并进行了手术切除。组织学检查和免疫染色结果显示为高级别 MPNST。结论头颈部多发性骨髓瘤可能表现为非特异性症状。诊断通常需要结合临床、病理和免疫组化研究。治疗包括手术全切除和辅助放疗。
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