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By Wendy Sherman and Jeffrey Raizer

Meningiomas are the most common intracranial tumors. Although most are benign, morbidity can be high, and in some cases the tumor is fatal. In this clinical article, Drs. Wendy Sherman and Jeffrey Raizer of Northwestern University review the pathophysiology, presentation, and treatment of this tumor, including new prognostic indicators in atypical and malignant meningiomas.

Key points

  • In addition to meningioma formation, NF2 inactivation also has been associated with tumor progression to higher grades.
  • There are several other pathways that are involved in meningioma formation and progression, including the Jak-STAT-3 pathway, Notch signaling pathway, and overexpression of SPARC protein.
  • Papillary and rhabdoid meningiomas are rare variants and have an aggressive clinical course and higher rates of recurrence, metastases, and mortality.
  • Sixty percent of benign meningiomas are associated with peritumoral brain edema.
  • Degree of edema has a statistically significant positive correlation with higher recurrence rates, which suggests that edema should be used as a prognostic factor.
  • Skull involvement was associated with an increased rate of disease progression and decreased survival.
  • New genomic analyses have implicated AKT1 and SMO pathways in the pathogenesis of non-NF2 associated skull base meningiomas, which may serve as potential therapeutic targets in the medical management of these challenging tumors.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited