Meningioangiomatosis in a patient without von Recklinghausen' disease

Abstract

✓ Meningioangiomatosis is a rare benign tumor of the central nervous system. Most cases are associated with von Recklinghausen' neurofibromatosis. The case of a 9-year-old boy with a 6-year history of temporal lobe epilepsy is reported. Computerized tomography revealed a hyperdense lesion in the region. The lesion was avascular on angiography. Magnetic resonance imaging showed a large well-demarcated abnormality with hyper- and hypodense regions on T2-weighted images. The abnormality did not extend into the white matter. Histological sections of the biopsy specimen disclosed an organoid transcortical lesion composed of interlacing finger-like fascicles of vascularized fibromeninges. Most fascicles exhibited one or more central slit-like capillaries cuffed by fibroblasts and invaginated meningeal tissue arranged in a parallel or concentric manner. Typical psammoma bodies were evident throughout the lesion, as well as in the intervening, mildly gliotic cerebral cortex. Proliferative meningothelial cells were present in the overlying leptomeninges. The lesion in this case was a hamartoma rather than a neoplasm.