Surgical outcome of isolated benign peripheral nerve sheath tumors without neurofibromatosis

IF 0.7 Q4 CLINICAL NEUROLOGY Egyptian journal of neurosurgery Pub Date : 2024-05-28 DOI:10.1186/s41984-024-00297-2
Mohammud Ahmud Salim, Hossam Elnoamany, Mohammed Adel Dorrah, Zahraa M. Mahdy, Ahmed Said Mansour
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Abstract

Benign peripheral nerve sheath tumors (BPNSTs) include schwannomas and neurofibromas. About 10% of soft tissue sarcomas are malignant peripheral nerve sheath tumors (MPNSTs), which are invasive and aggressive tumors. These can happen occasionally or after radiation exposure. Up to 90% of schwannomas are made up of differentiated neoplastic Schwann cells. Malignant transformation of schwannomas is rare. We collected the medical records of all patients (including their family histories), performed comprehensive physical and neurological assessments, and checked for the presence of a Tinel-like sign, as well as screening for neurofibromatosis (NF) signs. Magnetic resonance imaging (MRI), nerve conduction studies, and ultrasound were done for all cases. We have operated on 21 patients with age range 29–52 years. The mean age was 39.4 years. 14 of these patients were females and 7 were males. The presenting symptoms were just swelling at nerve site in 14 patients, spontaneous pain at the nerve sites in 7 patients, and sensory deficit at the nerve distribution sites in 9 patients. The other 12 patients were sensory intact and only 7 patients had motor deficit. Postoperatively all patients had improved motor and sensory deficit and none of intact patients were worsened. Preoperatively we did MRI to show important nearby vascular structure anatomical abnormalities and we ordered nerve studies to all patients that showed abnormalities, which was only in 9 patients. Gross total resection was done in 18 patients and other 3 cases had partial resection to avoid sensory and motor deficits. The pathological analysis revealed 11 schwannomas and 10 neurofibromas. With 1 year follow up there was no recurrence in any patients. Benign pheripheral nerve sheath tumours are safely resected without increased sensory and motor deficits after surgery and with improve clinical outcome with no recurrence on follow up.
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无神经纤维瘤病的孤立性良性周围神经鞘瘤的手术疗效
良性周围神经鞘瘤(BPNST)包括分裂瘤和神经纤维瘤。大约 10% 的软组织肉瘤是恶性周围神经鞘瘤(MPNST),这是一种侵袭性和侵袭性肿瘤。这些肿瘤可能偶尔发生,也可能在暴露于辐射后发生。多达 90% 的裂神经瘤由分化的肿瘤性许旺细胞组成。分裂瘤的恶性转化非常罕见。我们收集了所有患者的病历(包括他们的家族史),对他们进行了全面的身体和神经系统评估,检查是否存在Tinel样体征,并筛查神经纤维瘤病(NF)体征。所有病例都进行了磁共振成像(MRI)、神经传导检查和超声波检查。我们已为 21 名患者实施了手术,他们的年龄在 29-52 岁之间。平均年龄为 39.4 岁。其中 14 例为女性,7 例为男性。14 名患者的主要症状为神经部位肿胀,7 名患者为神经部位自发疼痛,9 名患者为神经分布部位感觉缺失。其他 12 名患者感觉正常,只有 7 名患者有运动障碍。术后,所有患者的运动和感觉障碍均有所改善,完好无损的患者无一恶化。术前,我们做了核磁共振成像,以显示附近重要的血管结构解剖异常,并对所有显示异常的患者进行了神经检查,仅有9名患者出现异常。我们对 18 例患者进行了全切,另外 3 例进行了部分切除,以避免感觉和运动障碍。病理分析显示有 11 个分裂瘤和 10 个神经纤维瘤。经过一年的随访,所有患者均未复发。良性外周神经鞘瘤可安全切除,术后不会增加感觉和运动障碍,临床疗效较好,随访期间无复发。
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