plexiform neurofibroma


plex·i·form neu·ro·fi·bro·ma

a type of neurofibroma, representing an anomaly rather than a true neoplasm, in which the proliferation of Schwann cells occurs from the inner aspect of the nerve sheath, thereby resulting in an irregularly thickened, distorted, tortuous structure; in some instances, the process extends along the course of the nerve and may eventually involve the spinal roots and the spinal cord; seen most frequently in neurofibromatosis. Synonym(s): fibrillary neuroma, plexiform neuroma

plexiform neurofibroma

A benign, non-encapsulated tumour composed of proliferating axons, Schwann cells, fibroblasts and perineurial cells.
 
Types
• Plexiform neurofibromas may cause a marked deformation and enlargement of a body part or region, an event known as elephantiasis neuromatosa.
• Cutaneous and subcutaneous neurofibromas are seen in type-1 neurofibromatosis (NF), are non-circumscribed, thick, irregular, may cause disfigurement by entwining critical structures, and are usually associated with type-1 NF.
 
Locations
Orbit, neck, back, inguinal region.

plex·i·form neu·ro·fi·bro·ma

(plek'si-fōrm nūr'ō-fī-brō'mă) A type of lesion, representing an anomaly rather than a true neoplasm, in which the proliferation of Schwann cells occurs from the inner aspect of the nerve sheath; seen most frequently in neurofibromatosis.
Synonym(s): plexiform neuroma.