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Meningioangiomatosis: clinical, radiologic, and histopathologic correlation
R N Aizpuru1, R M Quencer, M Norenberg
1Department of Radiology, University of Miami School of Medicine, FL 33136.
Abstract:
Meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion of the leptomeninges; MA has been considered to be a forme fruste of neurofibromatosis. A review of pathology records for patients with MA who were seen between 1970 and 1989 at the authors' institutions revealed four patients (three male and one female; aged 2.5-21.0 years; mean, 10.8 years) with a history of seizures but without the stigmata or family history of neurofibromatosis. Three patients had undergone either computed tomographic (CT) or magnetic resonance (MR) imaging studies. All patients had undergone craniotomies to obtain tissue for pathologic analysis; a peripheral, leptomeningeal lesion was found in all four patients. At CT in two patients, the lesions were most consistent with calcification. At T2-weighted MR imaging in one patient, the lesion demonstrated a hyperintense periphery with associated edema of the white matter. Histopathologic examination demonstrated characteristic features of MA--cortical meningovascular fibroblastic proliferation and leptomeningeal calcification. The accurate diagnosis of MA is important since MA is a benign, surgically correctable cause of seizures.
Insights
Meningioangiomatosis (MA) is a rare, benign brain lesion. Accurate diagnosis is crucial as it is a surgically correctable cause of seizures, often presenting without typical neurofibromatosis signs.
Area of Science:
- Neurology
- Pathology
- Neurosurgery
Background:
- Meningioangiomatosis (MA) is a rare, benign leptomeningeal lesion.
- MA is sometimes considered a forme fruste of neurofibromatosis.
- Its distinct presentation and diagnostic criteria require clarification.
Purpose of the Study:
- To review cases of meningioangiomatosis.
- To analyze clinical, imaging, and histopathologic findings.
- To emphasize the importance of accurate diagnosis for surgical intervention.
Main Methods:
- Retrospective review of pathology records (1970-1989).
- Analysis of four patients with MA and seizures, without neurofibromatosis stigmata.
- Review of computed tomography (CT), magnetic resonance (MR) imaging, and histopathology.
Main Results:
- Four patients (2.5-21.0 years) with MA and seizures were identified.
- Imaging showed calcification (CT) and hyperintense periphery with edema (MR).
- Histopathology confirmed meningovascular fibroblastic proliferation and calcification.
Conclusions:
- Meningioangiomatosis can present as a cause of seizures without neurofibromatosis.
- Accurate diagnosis via histopathology is essential.
- MA represents a benign, surgically correctable etiology of seizures.
