Neurofibroma is a benign tumor of the peripheral nerve sheath. It arises due to the abnormal proliferation of Schwann cells, perineural cells, and endoneurial fibroblasts. Several types of pathology are identified, including localized, plexiform, and diffuse types.1,2 Neurofibromatosis type 1 (NF1) is a neurocutaneous, autosomal dominant disorder.3 NF1 affects approximately 1/2700 newborns, with variable presentation and disease severity.3 The disease manifestations are café-au-lait macules (CALMs), neurofibromas, skin-folding freckling, iris hamartomas (Lisch’s nodules), optic pathway gliomas, and skeletal deformities.1,3 Plexiform neurofibroma is a benign tumor of the peripheral nerves (World Health Organization grade I) and an unusual variant of neurofibroma that arises from a proliferation of all parts of the nerve. Plexiform neurofibroma is essentially pathognomonic of NF1.2,4–6 This tumor has a significant risk of eventual malignant transformation.7 The possibilities of malignancy and recurrence are the main reasons for long-term, close follow-up.8–13 Plexiform neurofibromas are an uncommon type of NF1 developed from multiple nerves as bulging and deforming masses that also involve connective tissue and skin folds, also known as “bags of worms.”13–17 We report a case of recurrent plexiform neurofibroma, originating from the peripheral nerves, which also presented with typical signs of NF1 disease.
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