Growing Suspicion

The patient underwent repeat MRI in May 2014 in preparation for surgical resection of the mass. This showed complete enhancement of the skull base with the appearance of meningioma creeping down both intracranial optic canals and considerable tumor in the right orbit along the optic nerve and some tumor along the left optic nerve near the apex. He underwent right frontal skull base craniotomy with microscopic removal of suprasellar, right sphenoid wing, and skull based tumor. Intraoperatively, the tumor was found to be molded to the entire skull base as though carpeting had been laid over the structures including a heavy coating over the arteries. Pieces were removed and sent to pathology intraoperatively for examination. The preliminary description was highly atypical for meningioma and more likely to represent an inflammatory process. As the mass wrapped itself around the optic nerves and chiasm, it was not dissected off for fear of stripping the patient's own blood supply and the area was decompressed. Pathology showed marked granulomatous inflammation with multinucleated giant cells and central necrosis. No organisms were noted and AFB and GMS stains. This was felt to be consistent with meningeal sarcoidosis and the patient was referred to rheumatology for further management. He was placed on oral steroids as well as methotrexate with consideration of use of Remicade for long-term control. However, 3 months post-operatively he became short of breath and sustained a cardiac arrest, requiring defibrillation. Evaluation revealed popliteal DVT and bilateral PE's as well as radiographic evidence of intraseptal sarcoidosis. He underwent placement of an AICD and is currently on amiodarone and anticoagulation for nonsustained ventricular tachycardia. On ophthalmic follow up, his right eye remains counting fingers in the superotemporal quadrant and the left eye vision has returned to 20/20 with near-complete resolution of the visual field defect.