IntroductionPeripheral nervous system tumors, classified by the World Health Organization in 2020 as mesenchymal tumors, are benign in 95% of cases and include schwannomas and neurofibromas [1]. Plexiform schwannomas (PS), representing fewer than 5% of cases, display multifascicular intraneural and multinodular growth along a single nerve fascicle. These tumors typically appear in superficial cutaneous or subcutaneous nerves but can occasionally affect major nerves [2]. While benign, PS often recur locally and rarely transform into malignant peripheral nerve sheath tumors (MPNST). Plexiform neurofibromas, another rare variant, affect major nerves with a ”bag of worms” appearance and may cause deformities, with a 5% risk of malignant transformation [3]. Unlike plexiform neurofibromas, PS are linked to genetic syndromes like neurofibromatosis (NF) or schwannomatosis in only 5% of cases [2].
