Meningiomas: Overview and New Directions in Therapy

Nancy Wang, MD; Matthias Osswald, MD


Semin Neurol. 2018;38(1):112-120. 

In This Article


Although most meningiomas are classified as benign, studies have shown that survival is still shorter than in matched controls.[73–75] Survival for anaplastic meningiomas is significantly reduced, with a 10-year relative survival rate of 57.5%.[1]

Prognostic factors, including those influencing survival, are age, tumor size and location, extent of resection, mitotic index, molecular and histological features, and initial therapy.[1,75,76] The Simpson grading system, published in 1957, is a predictive model that uses the extent of resection to predict meningioma recurrence (Table 2).[77] Recent studies have had more conflicting findings regarding the prognostic importance of the extent of resection in the modern era.[78–80] Tumor location also plays a significant role in surgical resectability and therefore prognosis. Imaging characteristics such as low apparent diffusion coefficient on MRI may be additional predictors of progression or recurrence.[81]

Increasing knowledge of the genetic landscape of meningiomas has further improved prognostic understanding. Increased expression of MYCN, mutations in SMO and AKT1, deletions in the 9p21 region (including the CDKN2A gene), increased programmed death-ligand 1 (PD-L1) expression, gain of 1p, and deletion of 14q have all been associated with poor prognosis.[82–90] Recent studies have demonstrated that DNA methylation patterns could predict tumor recurrence even more accurately than the current WHO grading system.[27,91]