BRAIN AND NERVOUS SYSTEM: Edited by Marc SansonMolecular alterations in meningioma: prognostic and therapeutic perspectivesBirzu, Cristinaa; Peyre, Matthieub; Sahm, Felixc,d,eAuthor Information aService de Neurologie 2-Mazarin bService de Neurochirurgie, Sorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France cDepartment of Neuropathology, Institute of Pathology, University Hospital Heidelberg dClinical Cooperation Unit Neuropathology (B300), German Consortium for Translational Cancer Research (DKTK), German Cancer Research Center (DKFZ) eHopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany Correspondence to Felix Sahm, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 280, 69120 Heidelberg, Germany. Tel: +49 06221 56 37886; e-mail: Felix.Sahm@med.uni-heidelberg.de Current Opinion in Oncology: November 2020 - Volume 32 - Issue 6 - p 613-622 doi: 10.1097/CCO.0000000000000687 Buy Metrics Abstract Purpose of review To discuss recent advances in the meningioma biology and their clinical implications. Recent findings Meningioma is the most common primary intracranial tumor. Mostly benign, 20% of cases display an aggressive behavior despite best standard of care. The genetic landscape of meningiomas is divided according to NF2 mutational status. Although about 60% of meningiomas display NF2 mutations, the other share is more heterogenous. Mutations in TRAF7, SMO, v-akt murine thymoma viral oncogene homolog 1 (AKT1), PI3KCA and KLF4 are seen mostly in WHO grade 1 meningiomas. In higher grade meningiomas, mutations of the TERT promoter and deletions of CDKN2A/B emerge and have prognostic value. Moreover, mutations in DMD, BAP1 and PBRM1 have recently been discovered and are being further explored. DNA methylation subgroups offer valuable insight into meningioma prognosis and its implementation in clinical setting is under evaluation. Moreover, the study of distinct meningioma populations such as radiation-induced meningioma and progestin-associated meningioma may provide further insight into meningioma oncogenesis and potential therapeutic targets. Summary The mutational landscape of meningioma has expanded following the use of the new genetic sequencing approaches. Novel mutations have been characterized and reveal their prognostic and therapeutic applications. This improved understanding of meningioma biology has promising implications for novel treatment strategies. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.