Aggressive orbital optic nerve meningioma with benign microscopic features: a case report.

Primary optic nerve meningiomas occur at lower ages than meningiomas arising from the coverings of the brain and spinal cord. Here we report the case of a 20-year-old female with an aggressive orbital meningioma referred to the Ophthalmology Department of the Farabi Hospital in Tehran. The patient had a history of orbital meningioma from 10 years ago and several surgical resections due to tumor recurrence during these 10 years. On admission, the patient had a large orbital mass and severe proptosis. MRI images revealed a large left orbital mass with optic nerve involvement and extension to the left maxillary sinus, pterygoid fossa and the dura in the floor of the anterior fossa. Fine-needle aspiration cytology of the mass confirmed tumor recurrence. The patient first received radiotherapy due to the inoperable mass, and the tumor was resected 1.5 month later. Microscopic study showed meningotheliomatous meningioma with extensive involvement of the optic nerve and invasion of the optic disc, sclera and choroid. The interesting aspect of this case was the aggressive behavior of the tumor with intraocular invasion, despite its benign histopathological features, which led to wide exenteration of the eye together with resection of the upper and lower lids.