Diagnosis and management of intracranial malignant peripheral nerve sheath tumors.

Abstract

Intracranial malignant peripheral nerve sheath tumors (MPNSTs) are rare and generally carry a poor prognosis. We have analyzed our experience with MPNSTs and conducted a review of the literature in an attempt to identify a rational approach to the management of these tumors. Eight patients underwent surgical treatment for intracranial MPNSTs during a 10-year period from 1996 to 2005. The general strategy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for residual tumor. Chemotherapy was not used in this group. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively. Six of the eight patients underwent primary operations; two of the eight patients had previously undergone other transcranial surgery operations. Total tumor resection was achieved in five patients. At this time, two have been recurrence-free for 3.5 and 5 years, respectively, and the other three patients had a mean postoperative survival of 7 months. There was one case of near total (>90%) and two cases of partial (<90%) tumor removal; the postoperative survival rate was 4, 4, and 2 months, respectively. Only two patients in our group accepted radiotherapy after surgery; one survived only 4 months and the other has been recurrence-free for 5 years. MPNSTs are fast-growing, invasive tumors with rather unsatisfactory outcomes. Total surgical resection seems to be the most effective therapeutic method, and radiotherapy may play a role in local control.