Large Orbital Tumor in a Patient with Neurofibromatosis Type 2

Neurofibromatosis type 2 (NF2) is a separate entity from Neurofibromatosis type 1 (NF1) or von Recklinghausen’s disease, and is much less frequent than NF1. Vestibular schwannomas are the hallmark lesion, affecting 95% of individuals and typically occur bilaterally. Schwannomas commonly occur on other nerves intracranially and in the spinal compartment, along with meningiomas, ependymomas, and gliomas. Ophthalmologic abnormalities are present in the majority of NF2 patients, including cataracts, retinal changes, optic nerve sheath meningiomas and other optic pathway tumors. These tumors are sometimes large and can induce significant proptosis. We report the case of a large orbital meningioma responsible for severe proptosis in a patient with NF2.