Surgical Management of Pediatric PNST

Most peripheral nerve sheath tumors in children are benign lesions, associated with neurofibromatosis (neurofibromas) or schwannomatosis (schwannomas) in most cases. Most sporadic tumors are schwannomas. Indication for surgical resection is generally dictated by neurological deficits or pain. Intracapsular resection with preservation of the nerve harboring the tumor is the treatment of choice. Postoperative neurological deterioration is rare. The ubiquitous presentation of tumors should prompt for interdisciplinarity of different surgical disciplines. The goal of surgical treatment is the improvement of symptoms with the conservation of neurological function. Plexiform neurofibromas (PNF) pose a diagnostic and therapeutic challenge. Cutaneous PNF should be resected when showing growth or cosmetic impairment. Infiltration of surrounding tissue may render these tumors unresectable. PNF of peripheral nerves may also appear diffuse with local infiltration, prompting incomplete resection and risk of recurrence. Malignant peripheral nerve sheath tumors usually arise from PNF. Overproportional growth, significant pain, and suspicion of malignancy via imaging studies should prompt for adaption of pre- and intraoperative workflow. The treatment goal in these tumors is oncological, usually consisting of radical resection and often adjuvant therapy.