Abstract

Meningiomas are a heterogeneous group of tumors, defined histo-pathologically by World Health Organization (WHO) grading. The WHO grade of meningiomas does not always correlate with clinical aggressiveness. Despite maximal surgical resection and adjuvant radiation, a subset of tumors are clinically aggressive; displaying early recurrence and invasion. Current methods for identifying aggressive meningiomas solely focus on genomics, proteomics, or epigenetics and not a combination of all for developing a real-time clinical biomarker. Improved methods for the identification of these outlying tumors can facilitate better classification and potentially adjuvant treatment planning. Understanding the pathways of oncogenesis using multiple markers driving aggressive meningiomas can provide a foundation for targeted therapies, which currently do not exist.

Highlights

  • Meningiomas are the most common central nervous system tumor, with an incidence of approximately 8 per 100/000 people [1]

  • Meningiomas are a heterogeneous group of tumors and are currently graded according to the World Health Organization (WHO) classification, last revised in 2016

  • Tumors with Krüppel-like factor 4 (KLF4) mutations are associated with larger peritumoral brain edema, localize to the anterior and middle cranial skull base, and when present with tumor necrosis factor receptor associated factor 7 (TRAF7) mutations are predictive of a secretory meningioma phenotype [24, 40]

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Summary

Introduction

Meningiomas are the most common central nervous system tumor, with an incidence of approximately 8 per 100/000 people [1]. We review the current understanding of the meningioma genomic, proteomic, and epigenetic landscape, and discuss translational efforts to identify patients at high risk of recurrence and tumors with targetable mutations that may lead to more efficacious medical therapies. Despite a revolution in the genomic landscape of meningiomas localizing to specific anatomical locations, alterations in high risk progression or recurrence tumors is lacking.

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