Purpose of review: Advances have been made in the treatment of primary optic nerve tumours. With a focus on the last few years' publications, recommendations for clinical management are being developed.
Recent findings: In low-grade optic nerve glioma, two divergent developments are observed: an increasing reluctance in treating such tumours because of reports about treatment toxicity (secondary tumours, moyamoya syndrome) and a steady and marked improvement both in radiotherapy and chemotherapy. Many reports on beneficial effects of radiotherapy on optic nerve meningioma have been published. Radiotherapy does not only preserve but in many cases even improves or restores visual function and has, therefore, become the therapy of choice in this tumour.
Summary: Establishing a treatment plan in cases of optic nerve glioma is difficult and must be made on an individual basis. Although both chemotherapy and radiotherapy can stabilize and sometimes improve vision in progressive tumours, chemotherapy is the preferred modality in children younger than 9 years and in patients with neurofibromatosis 1. In functionally progressive optic nerve meningioma with useful visual function, multifractioned stereotactic conformal radiotherapy is the treatment of choice.