The aim of the paper is to provide an overview of clinical, histo-logical and radiological features of the most common primary tumours of the optic nerve and optic nerve sheath, as well as to discuss available treatment options.
often affect patients with NF1, whereas meningiomas more often affect those with NF2. Unlike in children, optic nerve glioma in adults can be malignant. On the other hand, optic nerve sheath meningioma tends to be more malignant in children rather than in adults. The symptoms of optic nerve glioma and optic nerve sheath meningioma can be similar, including vision impairment and proptosis. The diagnosis is based on orbital imaging with magnetic resonance image (MRI) or computed tomography (CT). The management most often involves clinical observation. Surgery, radiation therapy or chemotherapy are only used in selected cases with the risk of optic chiasm or fellow optic nerve involvement.