Chemotherapy for Optic Nerve Glioma in A Child with Neurofibromatosis Type-1

The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be reserved only for patients with optic chiasm or tract involvement.