Clinical potential of meningioma genomic insights: a practical review for neurosurgeons

Neurosurg Focus. 2018 Jun;44(6):E10. doi: 10.3171/2018.2.FOCUS1849.


Meningiomas are among the most common intracranial pathological conditions, accounting for 36% of intracranial lesions treated by neurosurgeons. Although the majority of these lesions are benign, the classical categorization of tumors by histological type or World Health Organization (WHO) grade has not fully captured the potential for meningioma progression and recurrence. Many targeted treatments have failed to generate a long-lasting effect on these tumors. Recently, several seminal studies evaluating the genomics of intracranial meningiomas have rapidly changed the understanding of the disease. The importance of NF2 (neurofibromin 2), TRAF7 (tumor necrosis factor [TNF] receptor-associated factor 7), KLF4 (Kruppel-like factor 4), AKT1, SMO (smoothened), PIK3CA (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha), and POLR2 (RNA polymerase II subunit A) demonstrates that there are at least 6 distinct mutational classes of meningiomas. In addition, 6 methylation classes of meningioma have been appreciated, enabling improved prediction of prognosis compared with traditional WHO grades. Genomic studies have shed light on the nature of recurrent meningioma, distinct intracranial locations and mutational patterns, and a potential embryonic cancer stem cell-like origin. However, despite these exciting findings, the clinical relevance of these findings remains elusive. The authors review the key findings from recent genomic studies in meningiomas, specifically focusing on how these findings relate to clinical insights for the practicing neurosurgeon.

Keywords: AKT1; KLF4; NF2; PI3K = phosphoinositide 3-kinase; PIK3CA; POLR2; SCNA = somatic copy-number alteration; SMO; TRAF7; WHO = World Health Organization; genomics; mTOR = mammalian target of rapamycin; meningioma.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / genetics
  • Genomics / methods*
  • Humans
  • Kruppel-Like Factor 4
  • Meningeal Neoplasms / genetics*
  • Meningeal Neoplasms / surgery*
  • Meningioma / genetics*
  • Meningioma / surgery*
  • Mutation / genetics
  • Neurofibromin 2 / genetics
  • Neurosurgeons / education*
  • Neurosurgeons / trends
  • Smoothened Receptor / genetics


  • Biomarkers, Tumor
  • KLF4 protein, human
  • Kruppel-Like Factor 4
  • Neurofibromin 2
  • SMO protein, human
  • Smoothened Receptor