General | Imaging | Pathology | Surgery

General

• young to middle aged
• slow proptosis over yrs up to 4 mm
• occur in 1% of pts with NF1
• choroidal striae with hyperopia, usually no diplopia
• perineural spread and compression can cause pain

Imaging

Pathology

• benign
• usually superior orbit
• Antoni A (Verocay bodies, whorls and palisades)
• and B (myxoid with degeneration) patterns, no axons present
• S100 positive
• proliferations of Schwann cells encapsulated by perineurium

Surgery

• at surgery it is well encapsulated, yellow, and can be stripped away from nerves
• can recur but low malignant potential