Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses

Abstract

Introduction Peripheral nerve sheath tumours in children are a rare and heterogeneous group, consisting mostly of benign tumours as well as malignant neoplasms. Especially in the paediatric population, diagnostics and indication for therapy pose relevant challenges for neurosurgeons and paediatric neurologists alike. Most paediatric cases that need surgical intervention are associated to neurofibromatosis type 1 (NF1). Methods We retrospectively reviewed all paediatric cases treated at the Department of Neurosurgery in Tübingen between 2006 and 2017 for peripheral nerve sheath tumours. We analysed clinical signs, symptoms, histology, association to an underlying phacomatosis and sensory/motor function. Results Of the 82 identified patients, the majority had NF1 (76.8%). Nine children bore a sporadic tumour without underlying phacomatosis (11%), 8 had NF2 (9.8%) and 2 schwannomatosis (2.4%), A total of 168 surgical interventions were performed, and 206 tumours were removed. Indication for surgery was in most instances significant tumour growth (45.2%) followed by pain (33.9%). New deficits led to surgery in 12.5% of interventions; malignancy was suspected in 8.3%. Histopathology revealed mostly neurofibromas (82.5%), divided into cutaneous neurofibromas (10.7%), infiltrating plexiform neurofibromas (25.7%) and peripheral nerve-born neurofibromas (46.1%). 12.1% of tumours were schwannomas, 2.9% MPNST, 1.5% ganglioneuroma (n = 3) and 1 hybrid-neurofibroma and perineurinoma each. Leading symptoms, such as pain and motor and sensory deficits, improved after 125/166 interventions (74.4%), remained unchanged following 39 interventions (23.2%) and worsened in 4 occasions (2.4%). Conclusion Surgery is safe and effective for (neurofibromatosis associated) peripheral nerve sheath tumours in the paediatric population; however, management needs a multidisciplinary setting. We propose early surgical resection in paediatric patients with peripheral nerve sheath tumours with significant growth, or pain, or motor deficit, or suspected malignancy.

Management of neurofibromatosis type 2 and schwannomatosis associated peripheral and intraspinal schwannomas: influence of surgery, genetics, and localization

“…Data of 12 tumors of 10 pediatric patients (8 patients with NF2 and two with schwannomatosis) were part of a prior (published) study and were included in this cohort [ 13 ].…”

Treatment decisions and the use of MEK inhibitors for children with neurofibromatosis type 1-related plexiform neurofibromas

“…Indications for surgery include neurologic compromise or impact on vital structures, pain, and disfigurement, with the overall aim of surgery to reduce morbidity and improve QoL [1,30]. Approaches to surgical management of NF1-PN include complete resection and partial resection/debulking, whereby the goals of surgical management are to restore function, decrease pain, and improve PN-related disfigurement [36][37][38][39]. In many cases, this can only be partially achieved, by debulking, because complete resection is often not possible due to the size, location, and the network-like growth of PN [36][37][38][39].…”

“…Approaches to surgical management of NF1-PN include complete resection and partial resection/debulking, whereby the goals of surgical management are to restore function, decrease pain, and improve PN-related disfigurement [36][37][38][39]. In many cases, this can only be partially achieved, by debulking, because complete resection is often not possible due to the size, location, and the network-like growth of PN [36][37][38][39]. Although surgical treatment is indicated for symptomatic NF1-PN, complete removal is frequently challenging because of the significant risks of bleeding and neurologic damage, especially in deep-seated PN involving multiple nerves [1,30].…”