Alahmad Molham, Kharita Lubna, Alassaf Arige
Department of Otolaryngology-Head and Neck Surgery, Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria.
Department of Internal Medicine, Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria.
Ear Nose Throat J. 2024 Oct 23:1455613241293868. doi: 10.1177/01455613241293868.
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. 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. 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.
恶性外周神经鞘瘤（MPNSTs）是一种罕见的肿瘤，由外周神经鞘细胞发展而来，约占所有软组织肉瘤的5%至10%。头颈部区域的MPNSTs约占所有头颈部肉瘤的2%至6%，占所有MPNSTs的12%至19%，颞下窝是MPNSTs的罕见发病部位。起源于三叉神经的MPNSTs极为罕见。一名19岁女性，有6个月左侧面部疼痛及同侧感觉异常病史。检查发现左侧三叉神经第三支（V3）区域有面部感觉异常；计算机断层扫描和磁共振成像显示颞下窝有病变，遂行手术切除。组织学检查及免疫染色结果显示为高级别MPNST。头颈部区域的MPNSTs可能表现为非特异性症状。诊断通常需要结合临床、病理及免疫组化研究。治疗包括手术全切并辅以放疗。
