Optic gliomas and neurofibromatosis: neurosurgical management.

Abstract

Optic nerve gliomas occur in 5-15% of patients with neurofibromatosis; while many tumors are asymptomatic and stable, others cause significant morbidity and mortality. The availability of modern, noninvasive diagnostic modalities allows earlier diagnosis of this tumor, and even permits close observation as an alternative to therapeutic intervention in selected circumstances. Anterior tumors are best treated by observation, followed by surgical excision and radiation therapy if they enlarge and cause significant visual impairment. Posterior tumors are best treated by diagnostic biopsy and possible debulking, followed by radiation therapy. While chemotherapy appears a promising alternative to radiation therapy, insufficient experience has been obtained to draw any firm conclusions on the merit of this modality.