Hemangiopericytoma of the meninges of the optic nerve. A clinicopathologic report including electron microscopic observations.

A 61-year-old man complained of visual loss of his right eye following a systemic viral illness. At this time, complete ophthalmologic examination failed to disclose any abnormalities. Over the next months the patient developed dramatic loss of vision. Extensive workup, including CT scans, failed to clarify the underlying cause and a diagnosis of optic atropy following optic neuritis was made. The patient remained asymptomatic, but repeated CT scans depicted a fusiform enlargement of the intraorbital portion of the right optic nerve, three years after the onset of visual loss. The presumptive clinical diagnosis was optic nerve meningioma and the tumor was removed via a Krönlein procedure. Histopathologically, a highly vascularized intradural mass that led to compression atrophy of the optic nerve was present. The tumor was composed of fascicles of spindle-shaped cells that were interspersed among numerous, irregular vascular channels. Electron microscopically, the cells disclosed a lucent cytoplasm with a scarcity of organelles, prominent cytoplasmic filaments, numerous micropinocytotic vesicles and subplasmalemmal linear densities (hemidesmosomes). Poorly developed intercellular junctions were present between interdigitating cytoplasmic processes. The tumor cells surrounding the vascular units displayed a continuous basement membrane as well as all the features of well-differentiated pericytes. Away from vascular structures some tumor cells, which showed a focally discontinuous basement membrane, exhibited intermediate features between pericytes and fibroblasts. To the best of our knowledge, this is the first report of a hemangiopericytoma arising from the meninges of the optic nerve.