A lthough meningiomas are the most common primary brain tumor, the anaplastic variant is exceedingly rare, accounting for 1%-3% of all meningiomas. 16 These tumors display frankly anaplastic histology with an undeniably malignant clinical course. Median overall survival (OS) has been reported as 1.5 years, 28 with 5-year survival ranging from 47% to 61%. 6,11,30,32 Given the rarity of this meningioma variant and scarcity of literature, relatively little is understood with regards to the underlying pathogenesis and factors that impact clinical outcomes. While it has been well established that glioblastoma can present as either primary (i.e., arise de novo) or secondary (i.e., progress from a lower grade glioma), 4,23,37 the notion of progression has been more obscure in meningioma, although it would appear that anaplastic meningiomas can similarly develop through both mechanisms. 6,12,38 Regardless of pathogenesis, treatment strategies have often consisted of aggressive resection followed by radiotherapy, with various chemotherapies implemented in end-stage disease as no standard medical management abbreviatioNs CI = confidence interval; GTR = gross-total resection; MSKCC = Memorial Sloan-Kettering Cancer Center; OS = overall survival; STR = subtotal resection. submitted March 12, 2014. accepted October 15, 2014. iNclude wheN citiNg Published online April 10, 2015; DOI: 10.3171/2014.10.JNS14502. disclosure The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. results Overall, the median age at diagnosis was 59 years and 57% of patients were female. Most patients (38%) underwent 2 craniotomies (range 1-5 surgeries) aimed at gross-total resection (GTR; 59%), which afforded better survival when compared with subtotal resection according to Kaplan-Meier estimates (median overall survival [OS] 3.2 vs 1.3 years, respectively; p = 0.04, log-rank test). Twenty-three patients (62%) presented with apparently de novo anaplastic meningiomas. Compared with patients whose tumors had progressed from a lower grade, those patients with de novo tumors were significantly more likely to be female (70% vs 36%, respectively; p = 0.04), experience better survival (median OS 3.0 vs 2.4 years, respectively; p = 0.03, log-rank test), and harbor cerebral hemispheric as opposed to skull base tumors (91% vs 43%, respectively; p = 0.002). coNclusioNs Based on this single-center experience at MSKCC, anaplastic meningiomas, similar to glial tumors, can arise de novo or progress from lower grade tumors. These tumor groups appear to have distinct clinical behavior. De novo tumors may well be molecularly distinct, which is under further investigation. Aggressive GTR appears to confer an OS advantage in patients with anaplastic meningioma, and this is likely independent of tumor progression status. Similarly, those patients with de novo tumors experience a survival advantage likely independent of extent of resection.
Survival in patients treated for anaplastic meni...