JUL 17, 2013
The authors report on a rare case of optic nerve meningeal hemangiopericytoma, which they found may clinically simulate optic nerve sheath meningioma.
A 36-year-old woman presented with progressive loss of vision in the left eye for three years and rapid progression and painful protrusion of the eye for one month.
Clinical evaluation revealed no light perception, severe proptosis and hypoglobus, optic atrophy, and optociliary shunt vessels. Orbital imaging showed a well-defined heterogeneous intraconal mass partially encasing the optic nerve.
A clinical diagnosis of optic nerve sheath meningioma was made, and the tumor was completely excised along with enucleation, followed by postoperative adjuvant external beam radiotherapy.
At 15 months’ follow-up, there was no local recurrence. Histopathologically, the tumor was found to be arising from the optic nerve meninges with classical "stag-horn" pattern and abundant cellularity. Immunohistochemistry supported the histopathological diagnosis of hemangiopericytoma.
The authors write that the major challenge in management lies in making an accurate differentiation between aggressive hemangiopericytoma, meningioma and benign solitary fibrous tumor. Apart from its characteristic pathological features, immunohistochemistry helps differentiate this rare but clinically aggressive tumor from its benign counterparts. Given the tendency of meningeal hemangiopericytoma to recur or metastasize several years following initial treatment, it is important to deploy a multimodal management protocol for such tumors and meticulously follow them for several years.