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Optic Pathway Glioma




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


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