Chemotherapy and targeted therapies for meningiomas: what is the evidence?

<jats:sec> <jats:title>Purpose of review</jats:title> <jats:p>Although most meningiomas are slow growing tumors mainly controlled by surgery with or without radiotherapy, aggressive meningiomas that fail these conventional treatments constitute a rare situation, a therapeutic challenge and an unmet need in neuro-oncology.</jats:p> </jats:sec> <jats:sec> <jats:title>Recent finding</jats:title> <jats:p>Mutational landscape in recurrent high-grade meningiomas includes mainly <jats:italic toggle="yes">NF2</jats:italic> mutation or 22q chromosomal deletion, whereas telomerase reverse transcriptase promoter, <jats:italic toggle="yes">BAP-1</jats:italic> and <jats:italic toggle="yes">CDK2NA</jats:italic> mutations were also found in aggressive meningiomas. Pi3K-Akt-mTOR pathway is currently the most relevant intracellular signaling pathway target in meningiomas with preliminary clinical activity observed. Assessment of drug activity with progression free survival rate at 6 months is challenging in regard to meningioma growth rate heterogeneity, so that 3-dimensional growth rate before and during treatment could be considered in the future to selected new active drugs.</jats:p> </jats:sec> <jats:sec> <jats:title>Summary</jats:title> <jats:p>Despite a low evidence level, some systemic therapies may be considered for patients with recurrent meningioma not amenable to further surgery or radiotherapy. In recurrent high-grade meningioma, everolimus-octreotide combination, bevacizumab, sunitinib and peptide receptor radionuclide therapy exhibit a signal of activity that may justify their clinical use. Despite a lack of clear signal of activity to date, immunotherapy may offer new perspectives in the treatment of these refractory tumors.</jats:p> </jats:sec>