Optic nerve glioma - External and Internal Eye
Optic nerve gliomas are mostly seen in the pediatric population in the first decade of life. Gliomas in general are the most common CNS tumor in pediatric patients, and optic nerve gliomas make up 2%-5% of all gliomas. About 30%-50% of optic nerve gliomas in children are associated with neurofibromatosis type 1 (NF1). Optic nerve gliomas can be rarely found in adults. They almost exclusively present in males with a much more rapid progression and a poor prognosis.
Pediatric patients will present with a slow, progressive visual decline. In children, these tumors are typically slow growing. As the tumor progresses, a unilateral, painless proptosis can be seen, and patients may have total or partial vision loss at that time. Often, an afferent pupillary defect is present.
The most important risk factor for optic nerve gliomas is NF1, which is an autosomal dominant disorder. Approximately 10%-15% of NF1 patients will have optic nerve gliomas. Tumors associated with NF1 generally have the best prognosis. Sporadic tumors are more aggressive and have a higher chance of tumor progression and visual decline.
C72.30 – Malignant neoplasm of unspecified optic nerve
254976006 – Optic nerve glioma
Differential Diagnosis & Pitfalls