Management of primary spinal intraosseous malignant peripheral nerve sheath tumor

English Keywords:Malignant peripheral nerve sheath tumor  Primary  Spinal neoplasms  Diagnosis  Treatment

English Abstract:

  【Abstract】 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.