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 ?
Two or more neurofibromas or one plexiform neurofibroma.
Xanthoma, nevus sebaceous, skin tag, plexiform neurofibroma, lymphangioma, haemangioma, and focal dermal hypoplasia (Goltz syndrome) [1][2][3][4][5][6] should be considered where histology helps in the differentiation of these conditions.
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.
About 10% of NF1 patients will develop MPNST, which usually evolves from a benign--but often large and disfiguring--tumor called a 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.
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.
Pathologic evaluation of the nodules was performed to rule out the presence of a plexiform neurofibroma.
The 7 clinical criteria used to diagnose NF1 are six or more cafe-au-lait spots, Axillary or inguinal freckles, two or more typical neurofibromas or one plexiform neurofibroma, Optic nerve glioma, two or more iris hamartomas (Lisch nodules), Sphenoid dysplasia or typical long-bone abnormalities such as pseudarthrosis and first-degree relative with NF1 patients.
Other less common clinical features of NF1 include plexiform neurofibroma, skeletal abnormalities (short stature, scoliosis, long bone dysplasia, and osteopenia/ osteoporosis), Lisch nodules (iris hamartomas), neurocognitive deficits, cardiovascular abnormalities, and optic pathway gliomas.
Among them are the cell biology of phagocytosis, mechanism of function and disease of natural and replacement heart valves, the pathology of demyelinating diseases, the pathogenesis of mixed-lineage leukemia, tuberculosis pathogenesis and immunity, and the pathogenesis of plexiform neurofibroma.