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Topic Completed: 1 May 2016

Minor changes: 20 May 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Meningioangiomatosis

Jesse L. Kresak, M.D.
Page views in 2020: 814
Page views in 2021 to date: 754
Cite this page: Kresak JL. Meningioangiomatosis. website. Accessed December 8th, 2021.
Definition / general
  • Rare, benign, hamartomatous lesion of children and young adults characterized by leptomeningeal and meningovascular proliferation
  • Presents with seizures and headaches (or asymptomatic)
  • Mean age 21 - 28 years, range 1 - 70 years
  • 25% associated with neurofibromatosis II
  • May be associated with other developmental anomalies (Clin Neuropathol 2013;32:37, Pediatr Dev Pathol 2014;17:292)
Radiology description
Case reports
Gross description
  • Thick and opaque leptomeninges with abnormal vessels but no evidence of neoplasia
Microscopic (histologic) description
  • Often with plaque or rind-like meningothelial proliferation at cortical surface with tongue-like projections into underlying gray matter, predominantly in a perivascular pattern
  • Also increased cortical vascularity
  • Often leptomeningeal calcification
  • Variable psammoma bodies and osteoid
  • May have entrapped reactive glial tissue or neurons
  • Also variable neurofibrillary tangles
  • No / rare mitotic activity, no necrosis, no marked pleomorphism
Cytology description
  • Numerous thin walled capillaries, bland spindle cells, occasional large cells with prominent nucleoli, variable meningothelial whorls and neurons (Acta Cytol 2001;45:1069)
Positive stains
Negative stains
Differential diagnosis
  • Atypical meningioma: increased mitotic activity and either increased cellularity, high N/C, prominent nucleoli, sheet-like growth or necrosis
  • Desmoplastic infantile astrocytoma: prominent desmoplastic stroma with neuroepithelial cells
  • Invasive meningioma: infiltrative growth into brain parenchyma with destruction, no prominent vascular component
  • Schwannoma: encapsulated, Schwann cells in palisading or myxoid patterns, strong S100+, EMA-
  • Vascular malformation: Sturge-Weber syndrome or arteriovenous malformation, no perivascular meningothelial elements
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