Optic nerve glioma (OPG) is a rare tumor in children and adolescents. It comprises 1–5% of central nervous system tumors. It can be sporadic or associated with the neurofibromatosis 1 (NF1) gene. These are usually slow-growing tumors and may remain localized to the optic nerve or can have encroached upon adjoining structures like optic chiasma, opposite optic nerve, and hypothalamus. So, there may be decreased or loss of vision, proptosis, focal neurological symptoms, precocious puberty, and short stature. Due to the involvement of these critical structures, its treatment should be based on multidisciplinary consensus. The treatment modalities include surgery, RT, and chemotherapy. The aim of the treatment should be to preserve vision. However, the timing and selection of optimal treatment modalities are always a clinical dilemma. Recently, there have been promising results with newer techniques of radiotherapy and chemotherapy.
Part of the book: Radiation Oncology