The purpose of this study is to summarize our experience in managing patients with an atypical or malignant meningioma at our institution, with a specific focus on determining the prognostic factors for treatment outcome. We reviewed the records of 126 patients with atypical or malignant meningiomas from January 2001 to August 2011. Data collected included gender, age, Karnofsky Performance Scale (KPS) score, pathology results, cleavability, and bone invasion. The symptoms and signs were recorded for further outcome analysis. There were 37 malignant meningiomas and 89 atypical meningiomas. Total resection (Simpson grade I-II) was achieved in 80.9% of atypical patients (n = 72) and 67.6% of malignant patients (n = 25). Forty patients (44.9%) in the atypical group underwent radiotherapy after surgery, while 26 (70.2%) patients underwent radiotherapy in the malignant group. The median follow-up duration was 25 months. Patients with a secondary tumor had a much shorter progression-free survival (PFS) than those with a primary tumor in the malignant group. The malignant meningioma group had lower overall survival. Progression-free survival for patients in the malignant group who received postoperative radiotherapy was longer than that for those who did not receive radiotherapy. In conclusion, total resection of the tumor was important because patients with a secondary tumor were much more likely to have recurrence than patients with a primary tumor in the atypical and malignant meningioma groups. Also, radiotherapy should be performed after surgery for a malignant meningioma.
Object Intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNSTs) are rarely reported because of their extremely low incidence. Knowledge about these tumors is poor. In this study the authors aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial and intraspinal MPNSTs. Methods Among 4000 cases of intracranial and intraspinal PNSTs surgically treated between 2004 and 2011 at Beijing Tiantan Hospital, cases of MPNST were chosen for analysis and were retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed. Results Malignant PNSTs accounted for 0.65% of the entire series of intracranial and intraspinal PNSTs. Twenty-four (92.3%) of these 26 MPNSTs were primary. Radiologically, 26.9% (7 of 26) of the MPNSTs were misdiagnosed as nonschwannoma diseases. Twenty-one patients were followed up for 1.5 to 102 months after surgery. Twelve patients experienced tumor recurrence, and median PFS was 15.0 months. The 2- and 3-year PFS rates were 47.7% and 32.7%, respectively. Five patients died of tumor recurrence, and median OS was not available. The 2- and 3-year OS rates were 74.7% and 64.0%, respectively. Univariate analysis revealed that female sex, total tumor removal, and primary MPNSTs were significantly associated with a better prognosis. Multivariate analysis revealed that only total removal was an independent prognostic factor for both PFS and OS. Conclusions Malignant PNST within the skull or spinal canal is a rare neoplasm and is seldom caused by benign schwannomas. Radiologically, intracranial or intraspinal MPNST should be differentiated from meningioma, chordoma, fibrous dysplasia of bone, and ear cancer. Total resection whenever possible is necessary for the prolonged survival of patients, especially males.
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