Abstract

Meningiomas represent approximately 40% of all primary tumors of the central nervous system (CNS) and, based on the latest World Health Organization (WHO) guidelines, are classified into three grades and fifteen subtypes. The optimal treatment comprises gross total tumor resection. The WHO grade and the extent of tumor resection assessed by the Simpson grading system are the most important predictors of recurrence. Atypical meningiomas, a grade 2 meningioma, which represent almost a fifth of all meningiomas, have a recurrence rate of around 50%. Currently, different histopathologic, cytogenetic, and molecular genetic alterations have been associated with different meningioma phenotypes; however, the data are insufficient to enable the development of specific treatment plans. The optimal treatment, in terms of adjuvant radiotherapy and postoperative systemic therapy in atypical meningiomas, remains controversial, with inconclusive evidence in the literature and existing studies. We review the recent literature to identify studies investigating relevant atypical meningioma biomarkers and their clinical application and effects on treatment options.

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