Meningioangiomatosis: a case report and review of the literature

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22-year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electroencephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.