Optic Pathway Glioma

FACTS

  • 5% of primary intracranial pediatric tumors
  • can occur with NF1 (more indolent) or sporadically (more aggressive)
  • generally benign

HPI

visual disturbances
endocrinopathy
obstructive hydro sx

PHYSICAL EXAM

universal neuro exam
  • careful vision exam

IMAGING

  • Hydro: evaluate for intra-ventricular/exophytic extensions of tumor
  • Enhancement is important prognostic information (non-enhancing typically do not progress)
  • may causes a J-shaped sella/tuberculum (flattening of tuberculum becomes straight long part of J and sella is the curve)
A/P
Observation
Treatment indicated if progressing/deficits
  1. Chemo: generally first line vincristine/carboplatin
  1. Surgery: de-bulking can be appropriate, but GTR is very morbid (blindness)
  1. Radiation: also very morbid, generally avoided