Atypical meningiomas

Handb Clin Neurol. 2020:170:233-244. doi: 10.1016/B978-0-12-822198-3.00043-4.


Meningiomas are the most common primary CNS tumor in adults, representing a third of brain lesions. Their clinical presentation varies greatly, ranging from asymptomatic incidental tumor to fatal tumor. The majority of meningiomas are benign, and gross total resection can achieve very low recurrence rates, with radiation therapy or radiosurgery reserved for recurrences or residual tumor that grows during serial imaging. At the other end of the spectrum, malignant meningiomas, although comprising just 1% of meningiomas, have been recognized to exhibit aggressive behavior that ultimately proves lethal regardless of the extent of resection or whether adjuvant radiation and chemotherapy are utilized. Over the past 2 decades, there has been pathologic recognition of a third type of meningioma known as "atypical," with borderline histologic and clinical features between benign and malignant meningioma. Here we review the clinical features, treatment, and outcomes of atypical meningiomas, with a focus on the impact of extent of resection and radiation therapy on the long-term recurrence rate of completely and incompletely resected atypical meningiomas.

Keywords: Atypical meningioma; Radiation; Radiosurgery; Skull base; WHO grade II.

Publication types

  • Review

MeSH terms

  • Humans
  • Meningeal Neoplasms / diagnosis*
  • Meningeal Neoplasms / pathology
  • Meningeal Neoplasms / therapy
  • Meningioma / diagnosis*
  • Meningioma / pathology
  • Meningioma / therapy*
  • Neurosurgical Procedures
  • Radiosurgery