Management of primary optic nerve meningiomas. Current status--therapy in controversy.

The diagnosis, pathology, and management of primary optic nerve meningiomas are described from a study of 55 patients collected from the author's own series and the files of the Registry of Ophthalmic Pathology at the Armed Forces Institute of Pathology. Diagnosis may be confusing in childhood, especially in the presence of neurofibromatosis when it must be differentiated from optic nerve glioma. The disease is especially aggressive and life-threatening in the young. Of 15 patients under age 20 years, four died from intracranial extension, five are alive with recurrence, and two without recurrence. In the age group of patients between 20 and 35 years, the disease becomes less aggressive but still life-threatening. Of 14 patients in this group, one died of intracranial extension, four are alive with recurrence and seven without recurrence. In the older groups, the condition becomes more indolent in its growth pattern. In this series, no patients over 35 years of age have died from their disease. Surgical excision of the optic nerve and tumor for diagnosis, followed by craniotomy with total removal by unroofing the orbit and optic canal is advocated in the young. If there is orbital spread, exenteration should be performed. Craniotomy and more radical therapy are only indicated if intracranial extension is demonstrated. Radiotherapy is reserved as an alternative to surgery in the older age group.