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%|
|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