目的 探讨原发椎管内恶性周围神经鞘瘤(MPNST)的临床特点和治疗.方法 收集我院神经外科近10年来6例原发椎管内恶性周围神经鞘瘤手术患者资料,结合文献复习进行临床分析.结果 椎管内MPNST可累及脊髓任何节段,病变多发或呈丛状生长.6例患者行肿瘤全切除或近全切除术,术中肿瘤表现各异,术后病理诊断为恶性周围神经鞘瘤,部分患者术后进一步行辅助放疗.远期临床症状缓解明显,但肿瘤易复发.结论 原发于椎管内的恶性周围神经鞘瘤较为罕见,表现出恶性生物学行为,术前诊断困难,手术完全切除+术后辅助放疗治疗可能延缓疾病进展,但手术完全切除难度大,术后缺乏有效的辅助治疗方案,远期复发率高.%Objective To investigate the clinical characteristic and treatment of primary spinal malignant peripheral nerve sheath tumor ( MPNST). Methods The clinical data of 6 patients with primary spinal MPNST of our neurosurgery department in recent 10 years were analyzed with review of literature. Results The total or subtotal tumor resection were performed for each patient. The characteristics of the tumor vary from person to person. The diagnosis of MPNST was confirmed by postoperative pathology examination. Some went on for adjuvant radiotherapy. The symptoms relieved well after long-term of follow-up. But the recurrence rate of the tumor was relatively high. Conclusions Primary spinal MPNST has a low incidence. Preoperative diagnosis proves to be a challenge. Total tumor resection with adjuvant radiotherapy may be recommended. But given the malignant characteristic of the tumor total resection is difficult to achieve, and due to the lack of experience of adjuvant therapy the overall prognosis is poor.
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