CNS tumor
Meningeal tumors

Reviewer: Jesse L. Kresak, M.D. (see Authors page)

Revised: 11 May 2016, last major update May 2016

Copyright: (c) 2005-2016,, Inc.

PubMed Search: Meningioangiomatosis
Cite this page: Meningioangiomatosis. website. Accessed August 18th, 2017.
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
  • Two cases, 9 months to 33 years (Am J Surg Pathol 1999;23:872)
  • 7 year old girl with a 6 month history of partial complex seizures of increasing frequency (University of Pittsburg Case #92)
  • 11 year old boy with intractable seizures (Arch Pathol Lab Med 2003;127:e349)
  • 13 year old boy with sudden, unexpected death (Pediatr Dev Pathol 2005;8:240)
  • 15 year old boy with meningioangiomatosis and oligodendroglioma (Arch Pathol Lab Med 1996;120:587)
  • 16 year old boy with a genetic alteration (Am J Surg Pathol 2002;26:125)
  • 30 year old pregnant woman with fetal malformations (Pediatr Dev Pathol 2014;17:292)
  • 30 year old man with a frontal lobe mass (University of Pittsburg Case #354)
  • 33 year old man (Case Rep Oncol Med 2012;2012:296286)
  • Cortical blindness (Neurology 2013;81:511)
  • Treatment
  • Surgical excision cures most seizures (Clin Neurol Neurosurg 2013;115:1407)
  • 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
  • Microscopic (histologic) images

    Contributed by: Dr. Jesse Kresak, University of Florida (USA) - on Flickr:

    4x plaque or rind-like

    4x psammoma


    Images hosted on other servers:

    Prominent psammoma bodies;

    Psammoma bodies

    Degenerative changes

    Various images

    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
  • EMA
  • Progesterone receptor: variable
  • CD34: will highlight vascular elements
  • 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