Ectopic Meningioma Of The Orbit- A Report of Two cases

Saurbhi Khurana, Neelam Pushker, Dinesh Shrey, Seema Kashyap, Seema Sen, Mandeep S Bajaj

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Purpose: Ectopic meningiomas are extremely rare orbital tumors that do not originate from either the optic nerve or the intracranial meninges. Their origin remains a matter of controversy.

Methods: We describe two cases of ectopic meningiomas presenting as superomedial orbital masses along with their radiological and histopathological features.

Results: A 30 year old woman and a 40 year old man presented with progressive, painless proptosis. A firm mass was palpable in the superior orbit in both the cases; associated thickening of the superior orbital margin was found in the first case. Imaging in both cases revealed a well defined heterogenous superomedial orbital mass, separate from the optic nerve. Sclerosis and hyperplasia of the superior orbital rim was found in the first case. Both patients underwent anterior orbitotomy with debulking of the tumor. Histopathology and immunohistochemistry were consistent with the diagnosis of meningiotheliomatous meningioma in both cases. In both our patients, a recurrence was noted after 8 and 11 months respectively. A second surgery with complete excision of the tumor was done for both patients. No further recurrence was found.

Conclusions: Both cases presented with discrete masses without any connection with the optic nerve sheath or any intracranial extension. Though found to be locally invasive, surgical excision is usually possible in cases of ectopic meningiomas and the prognosis remains good.