CNS & pituitary tumors
Other tumors
Meningioangiomatosis
Author: Jesse L. Kresak, M.D.
Last author update: 1 May 2016
Last staff update: 20 May 2021
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Meningioangiomatosis
Table of Contents
Definition / general | Radiology description | Case reports | Treatment | Gross description | Microscopic (histologic) description | Cytology description | Positive stains | Negative stains | Differential diagnosisCite this page: Kresak JL. Meningioangiomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumormeningioangiomatosis.html. Accessed April 25th, 2024.
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 Pittsburgh: Seven Year Old Girl with Seizures)
- 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 Pittsburgh: A Male in His 30s with a Frontal Lobe Mass)
- 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
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
