脊柱原发恶性外周神经鞘膜瘤的诊断与治疗

Management of primary spinal intraosseous malignant peripheral nerve sheath tumor

目的：探讨脊柱原发恶性外周神经鞘膜瘤（malignant peripheral nerve sheat tumor,MPNST）的诊断和治疗方法。方法：1998年1月-2008年1月我科收治3例表现为椎体内病变的脊柱MPNST患者,均为女性,年龄分别为24、41、42岁。从出现症状到临床确诊的时间1～12个月,平均5个月。累及胸椎1例,腰椎2例。术前均行CT引导下病灶穿刺活检明确诊断为MPNST。均采用肿瘤包膜外分离、分块切除术。结果：3例患者术中平均出血4000ml,平均手术时间482min,2例患者肿物完全切除,1例患者肿物次全切除。术后症状均缓解。分别于术后3、4、30个月复发,2例复发后未再行手术治疗,其中1例行序贯放疗,分别于术后5个月、11个月死亡;1例复发后3次行肿瘤刮除术,末次术后行肿瘤疫苗治疗,末次术后随访20个月无复发。结论：脊柱原发MPNST罕见,影像学表现缺乏特异性,病灶穿刺取活组织行组织学及免疫组化检查是术前诊断的主要方法。手术可以缓解症状,但易复发,放疗对控制肿瘤复发效果不确切。

Objective：To investigate the diagnosis and management strategy of primary spinal intraosseous malignant peripheral nerve sheath tumor（MPNST）.Method：3 cases with primary spinal intraosseous MPNST treated in our department from January 1998 to January 2008 were reviewed retrospectively.All cases were women and aged 24,41,42 respectively.Intervals between the onset of symptoms and the confirmation of diagnosis ranged from 1 to 12 months with an average of 5 months.Thoracic spine was involved in 1 case and lumbar spine in 2 cases.All 3 patients underwent CT-guided biopsy and were confirmed with a clear diagnosis of MPNST.Surgical protocol of piecemeal resection was applied on all 3 patients.Result：The average intraoperative blood loss was 4000ml,and the average operation time was 482 minutes.En bloc tumor resection was achieved in 2 cases and subtotal resection in 1 case.After surgery,significant symptom improvement was noted in all cases,1 case underwent sequential radiotherapy and no chemotherapy was applied.Tumor recurrence was observed in all 3 cases at 3,4 and 30 months respectively after operation.Of these,two receive no reoperation and died 5 and 11 months respectively,while another case received treatment of tumor curettage for 3 times and tumor vaccine therapy with heat shock protein at final operation,no signs of recurrence was found at 20 months＇ follow-up.Conclusion：Primary spinal intraosseous MPNST is rare with no distinctive imaging features.Its diagnosis relies on pathological and immunohistochemical findings.Preoperative CT-guided biopsy is of great importance.Surgical resection is optional but the recurrence rate is high.Radiotherapy and chemotherapy remain unclear for the control of local recurrence and distant metastases.