Anterior Temporal Chordoid Meningioma Causing Compressive Optic Neuropathy

Purpose. Optic neuropathy associated with proptosis and ocular motility restrictions is suggestive of a compressive etiology. This raises concern for a mass lesion involving the orbit or orbital apex. Meningiomas, which account for 4% of all intraorbital tumors and 20 to 30% of all intracranial tumors, are capable of compressing critical anatomic structures within the orbit or orbital apex, and thereby impairing both afferent and efferent visual function. The purpose of this case report is to present a case of chordoid meningioma associated with compressive optic neuropathy. Methods. A 51-year-old woman presented with clinicopathological features pathognomonic of compressive optic neuropathy and orbital apex syndrome. Radiologic and neuropathologic evaluation revealed a large right anterior temporal atypical meningioma with chordoid features associated with frontal intraparenchymal edema. The therapeutic intervention consisted of orbitozygomatic craniotomy and radiotherapy. Results. Postoperatively, the patient progressed very well with considerable improvement of her visual and oculomotor function. The MIB-1 labeling index of 5 to 7% calls for close observation because of an increased probability of recurrence. Conclusions. This case highlights the importance of prompt diagnosis and treatment, because chordoid meningiomas exhibit a more aggressive biological behavior, with the potential to cause significant morbidity because of mass effect and higher risk of recurrence. To our knowledge, this is the first report of a rare case of a chordoid meningioma in the anterior temporal lobe that presented as a compressive optic neuropathy. The details of this case are presented with a review of relevant literature. (Optom Vis Sci 2011;88:645-651)