Abstract
Meningiomas are the most common adult primary intracranial tumor. Despite their higher incidence, there have not—until recently—been as many advances in understanding and managing meningiomas. Thus far, two broad classes of meningiomas have emerged on the basis of their mutational profile: those driven by neurofibromatosis 2 (NF2) inactivation and those with non-NF2 driver gene alterations, such as mammalian target of rapamycin and Hedgehog, Wingless/b-catenin, Notch, transforming growth factor-b receptor, mitogen-activated protein kinase, and phospholipase C pathway alterations. In addition to improvements in molecular diagnostics, advances in imaging are being studied to better predict tumor behavior, stratify risk, and potentially monitor for disease response. Management consists primarily of surgery and radiation therapy and there has been limited success from medical therapies, although novel targeted agents are now in clinical trials. Advances in imaging and understanding of the genetic makeup of meningiomas demonstrate the huge potential in revolutionizing the classification, diagnosis, management, and prognosis of meningiomas..
Highlights
Meningiomas are dural-based tumors that arise from arachnoid cap or meningothelial cells
Much work is being performed in the clinic, as well as in the fields of advanced imaging and genomics, to discover other features or phenomena that contribute to tumor growth and recurrence
Two broad classes of meningiomas have emerged on the basis of their mutational profile: those driven by neurofibromatosis 2 (NF2) inactivation and those with non-NF2 driver gene alterations, such as mammalian target of rapamycin and Hedgehog, Wingless (WNT)/b-catenin, Notch, transforming growth factor-b receptor (TGF-bR), mitogen-activated protein kinase (MAPK), and phospholipase C pathway alterations
Summary
Meningiomas are dural-based tumors that arise from arachnoid cap or meningothelial cells. This method could be used in conjunction with clinical and histologic grading scales to risk-stratify patients who require more aggressive upfront therapy with radiation[13] This idea was further reinforced by the Heidelberg, Germany group led by Sahm et al, who investigated genome-wide DNA methylation patterns of 497 meningiomas in a retrospective analysis and concluded that, compared with the current WHO classification, the DNA methylation-based meningioma classification is able to segregate meningiomas in more homogenous groups in terms of predicting tumor recurrence and prognosis[4]. A prospective phase II trial (ClinicalTrials.gov identifier: NCT00895622) that was conducted by the Radiation Therapy Oncology Group and that studied the effects of post-operative RT in intermediate-grade meningiomas (recurrent grade I with any extent of resection and gross totally resected grade II meningioma) reported a three-year PFS of 96% in patients who received RT23. A prospective NRG trial (ClinicalTrials.gov identifier: NCT03180268) is under way to study observation compared with radiation in patients with newly diagnosed gross totally resected WHO grade II meningiomas. An Alliance consortium group trial (ClinicalTrials.gov identifier: NCT02523014) is currently investigating targeted treatments in progressive meningioma on the basis of mutational status (NF2, AKT, and SMO)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
