5% of all childhood intracranial tumors are optic pathway gliomas1.
Generally low grade tumors, but follow a variable clinical course.
Summary of features of optic pathway gliomas (OPG):
| Feature | Proportion of all cases | |
| Proportion of OPG that occur in different age groups | < 18 months old | 25% |
| < 5 years old | 50% | |
| < 10 years old | 75% | |
| Median age to develop OPG | 5 years | |
| Tumor involves optic nerves alone | 20 - 25% | |
| Tumor involves optic chiasm (and or optic nerves) | 20 - 40% | |
| Tumor involves optic chiasm and hypothalamus | 30 - 60% | |
| Racial bias | None | |
| Gender bias | slight female | |
Neurofibromatosis type 1 (NF1)
- This genetic syndrome is associated with the development of optic pathway gliomas
- Autosomal dominant disorder characterized by abnormalities in ectodermal tissue growth.
- 25-40% of optic gliomas occur in children with NF-1, and of children with NF-1, about 10% develop optic gliomas.
Neurofibromatosis type 1 (NF1) at the Atlas of Genetics and Cytogenetics in Oncology and Haematology
Neurofibromatosis at DermAtlas
