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Clinical Features

      Usually occur in the dermal or subcutaneous tissues throughout the body
      People of any age can be affected, but seen most commonly in young adults
      Lesions may be localized, diffuse, or plexiform, the latter two having a strong association with NF1
      NF1, von Recklinghausen disease
      Autosomal dominant, chromosome 17
      Positive family history in most cases
      Multiple neurofibromas at different areas of the body
      Café-au-lait spots (hyperpigmented skin lesions)
      Lisch nodules (pigmented iris hamartomas)

Gross Pathology

      Well-defined fusiform lesion often in association with a nerve trunk
      Firm, gray-white cut surface
      Diffuse lesions show ill-defined, plaquelike thickening of the subcutaneous tissues
      Plexiform lesions are a multinodular conglomerate of lesions likened to a “bag of worms”


      Low to moderately cellular lesion composed of cells with wavy nuclei and eosinophilic cytoplasm interspersed with wisps of collagen
      Stroma …