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
- Non-encapsulated spindle cell proliferation
- Thin slandering nuclei (coma like)
- Fibrillary background
- May be plexiform
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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