Sylvian Fissure Meningioangiomatosis : A Case Report and Review of the Literature

Meningioangiomatosis (MA) is a rare malformation of the central nervous system. It has not been previously described in the literature. We report here the first case of MA involving the sylvian fissure in a 10-year-old boy. Clinicaly, he presented with a syncopal episode. Computed tomography revealed an enhancing hyperdense lesion in the right sylvian fissure. This was confirmed by magnetic resonance imaging of the brain with the lesion being isointense on T1-weighted images and hypointense on T2-weighted images and intense enhancement with gadolinium. The lesion was surgically removed and on histopathologic examination, the spindle cells were positive for vimentin on immunostaining. We suspect that it was not derived from arachnoid cap cells but originated from the mesenchmal cells. We summarize here the reported cases of MA and discuss the pathogenesis of this condition.