Association of stromal cells in development of oral neurofibromatosis and plexiform neurofibroma: a review of literature

Author: 
Ashok Vikey

Neurofibroma and plexiform neurofibroma are relatively uncommon nerve tissue tumors, affecting one person out of 3000 population. The pathogenesis of neurofibroma follows hereditary dominant trait, resulting through its protein neurofibrimin-1(NF-1), this is activated by dGAP and related domain (GRD), which stimulates to p21-RAS, and there is further damage of Schwann cells, which enhance growth and proliferation of tumor cells. At the same time other common factors get activated during growth of plexiform neurofibroma and NF-1, such as stromal components have exuberant response of EGFR, Stat3, stem cell population (SCP) and mast cells. All these biological substances govern growth and proliferation of these neural tumors. In spite of common pathogenesis, prognosis is different in both variants. The plexiform type shows comparatively unfavourable prognosis due to rapid and unpredicted proliferation. Pathogenesis of this nerve tissue tumor can reflect interventions, so its basic understanding can help in better prognosis.

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DOI: 
http://dx.doi.org/10.24327/ijcar.2021.24540.4868
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