Neurofibroma
- PMID: 30969529
- Bookshelf ID: NBK539707
Neurofibroma
Excerpt
Neurofibromas are the most prevalent benign peripheral nerve sheath tumor. Often appearing as a soft, skin-colored papule or small subcutaneous nodule, they arise from endoneurium and the connective tissues of peripheral nerve sheaths. Neurofibromas are comprised of Schwann cells, fibroblasts, perineural cells, and mast cells in a variably myxoid background. A mutation in the NF1 gene causes neurofibromas. There are three main types of neurofibromas: localized (most common), diffuse, and plexiform. Although the majority of neurofibromas occur sporadically and have an extremely low risk of malignant transformation, the plexiform type is pathognomonic for neurofibromatosis type 1 (NF 1). It carries an increased risk of malignant transformation. The complete excision of the lesion is curative.
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