MENINGIOANGIOMATOSIS - CLINICAL, RADIOLOGIC, AND HISTOPATHOLOGIC CORRELATION

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 of 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.