Neurofibromatosis type 1 (NF1) is a complex autosomal disorder caused by germline mutations in the NF1 gene, affecting approximately 1 in every 3000 individuals.2 Individuals with NF1 are predisposed to developing tumors originating from the embryonic neural crest.3 Among these typically benign tumors are plexiform neurofibromas (PNs), which are characterized by differentiated Schwann cells in a varied microenvironment composed of perineural-like cells, fibroblasts, vascular, and mast cells. This evidence concerns the gene NF1 and paraneoplastic neurologic syndrome.