Clinical analysis of 26 central nervous system malignant peripheral nerve sheath tumors

Objective To explore the diagnosis,comprehensive treatment strategies and prognosis for intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNST).Methods The investigators summarized 26 cases with MPNST which were performed by post-operation pathology from 2004 to 2011.By Log-rank and Cox model analysis,we analyzed the clinical features,radiological manifestations,pathological features,comprehensive treatment strategies and prognosis,and relevant literatures.Results Among the 26 cases,16 cases were male,10 cases were female,17 tumors were in cranial,9 were located in intraspinal.symptoms mainly included pain and impaired dysfunction of nerves.The follow-up data of 21 patients were collected from 1.5 to 102.0 months after surgery.Among these patients,5 patients had died due to tumor recurrence,7 patients were alive with tumor recurrence and 9 patients had no recurrence.Univariate analysis revealed that female gender,gross total resection,and primary MPNST were significantly associated with favorable prognosis(P ＜0.05).Multivariate analysis revealed only gross total resection to be an independent favorable prognostic factor (P =0.006).Conclusions Intracranial and intraspinal MPNST is a kind of rare neoplasm.The recurrence rate and mortality is higher.Gross total resection whenever possible is necessary for prolonged survivals.For patients with incomplete tumor resection,postoperative radiation therapy is recommended. Key words: Malignant peripheral nerve sheath tumors;  Central nervous system;  Diagnosis;  Comprehensive treatment;  Prognosis