Neurofibroma

Neurofibroma
Updated: 03/28/2020
© Jun Wang, MD, PhD

General features
  • Benign tumor with nerve sheath differentiation
  • If multiple, likely associated with neurofibromatosis type 1
  • Malignant transformation rare
  • Subtypes
Plexiform: Irregularly expanded nerve bundles with nodular appearance, prominent myxoid matrix; associated with NF1
Diffuse cutaneous: Large plaque like elevation, typically NF1-associated
Superficial cutaneous: Pedunculated nodules, solitary (sporadic) or multiple (likely NF1 associated)
Clinical presentations
  • Flesh-colored, soft nudule, solitary or multiple
  • Slow growing, painless
  • May have abnormal sensation (electric-like shock with light touch)
Key morphological features
Markers
  • Positive for S-100
  • May be focally positive for CD34 and Factor XIIIa
Genetic abnormalities
  • NF1 if neurofibromatosis
Treatment
  • Surgery if symptomatic

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