Diffuse neurofibroma
General: Most common in ages 10-30, in males & females, with a slight predeliction for the trunk, then head & neck, then limbs. Approximately 10% are associated with NF1. May become very large, but very rarely undergo malignant transformation.
Gross: Ill-defined plaque-like area of subcutaneous thickening, and may become very large.
Microscopic: Diffuse replacement of the dermis and subcutis by neurofibroma-like tumor (though the nuclei may appear more rounded), encasing and infiltrating rather than destroying, and with a fine fibrillary collagenous stroma. Foci of messnerian differentiation is identifiable in almost all cases. Nerves or nerve fibers within the lesion tend to be markedly hypertrophic and edematous. It's not uncommon to see pigmented dendritic cells. There may be rare multinucleate giant cells.
Differential Diagnosis:
- Dermatofibrosarcoma protuberans
- Neurofibroma (typical)
Stains:
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Cases:
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