Optic pathway glioma is most strongly associated with which condition?

• A. Neurofibromatosis type 1
• B. Down syndrome
• C. Marfan syndrome
• D. No known association

Answer: (A)

Optic pathway glioma in children is most strongly linked to Neurofibromatosis type 1. In NF1, loss of neurofibromin leads to unrestrained Ras signaling, which promotes glial cell growth along the optic nerve and chiasm. This genetic disruption makes optic pathway gliomas a relatively common tumor in NF1 patients, with many cases presenting as visual changes or be found incidentally. The other conditions don’t have a notable association with optic pathway glioma, so they’re not the best fit. Understanding this helps connect the genetic cause (NF1 affecting Ras regulation) with the tumor’s location along the optic pathway.