plexiform neurofibroma


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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.

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.
References in periodicals archive ?
In contrast to plexiform neurofibroma, it is not usually associated with neurofibromatosis type 1.
* Two or more neurofibromas or one plexiform neurofibroma.
Traditional optical (a) and confocal laser scanning (b) analyses showing well differentiated Schwann cells in a multinodular plexiform neurofibroma and their intense fluorescence.
(1) Six or more pigmentations greater than 5 mm before puberty and greater than 15 mm after puberty (cafe au lait) (2) One plexiform neurofibroma or more than two neurofibromas of any type (3) Axillary or inguinal freckling (4) Optic glioma (5) Two or more "Lisch" nodules (iris hamartoma) (6) Bone lesions (7) Presence of at least one of these clinical findings in first-degree relatives
Clinically, NF-1 is characterized by cafe-au-lait spots, freckling, skin neurofibroma, plexiform neurofibroma, bony defects, Lisch nodules, and tumors of the central nervous system.
In a retrospective review of 23 patients who underwent surgical resection of a plexiform neurofibroma pathologically diagnosed as either low-grade MPNST or ANF had disease-specific survival of 100% with a median follow-up of 47 months despite 78% (18/23) of patients having microscopically positive margins [26].
About 10% of NF1 patients will develop MPNST, which usually evolves from a benign--but often large and disfiguring--tumor called a plexiform neurofibroma.
The possible differential diagnosis in this case was plexiform neurofibroma. Plexiform neurofibroma is a variant of type 1 neurofibromatosis (Von Recklinghausen), where the diagnosis can be established when there are at least 2 of 7 criteria of National Institutes of Health (NIH), namely.3,8
We present a case of a 14-year-old patient with a personal history of VR and a surgical history of excision of a right hemicranial plexiform neurofibroma. No fibromas of the oral cavity or predictors of a difficult airway were found during airway exploration.