Benign Peripheral Non-cranial Nerve Sheath Tumors of the Neck

Authors: Carlos Suárez, Fernando López, Juan P. Rodrigo, William M. Mendenhall, Remco de Bree, Antti A. Mäkitie, Vincent Vander Poorten, Robert P. Takes, Stefano Bondi, Luiz P. Kowalski, Ashok R. Shaha, Veronica Fernández-Alvarez, Julio C. Gutiérrez, Nina Zidar, Carlos Chiesa-Estomba, Primoz Strojan, Alvaro Sanabria, Alessandra Rinaldo & Alfio Ferlito

Abstract

Benign peripheral non-cranial nerve sheath tumors are rare lesions, including both schwannomas and neurofibromas. These tumors arise from Schwann cells, and may originate from any peripheral, cranial, or autonomic nerve. Most of them are localized and sporadic but multifocal systemic forms can occur. Cervical sympathetic chain, brachial plexus, cervical plexus and spinal roots and nerves are the major nerve systems commonly affected. Dumbbell-shaped intra- and extradural tumors occur most commonly in the cervical spine, as well as purely extradural and paravertebral tumors. The management of these tumors has improved greatly owing to the developments in imaging techniques and surgical innovations such as endoscopically assisted approaches and robotic surgery. Microsurgical intracapsular excision of the tumor helped by the use of intraoperative fluorescent dyes and intraoperative neurophysiological monitoring minimize postoperative neural deficit, since most schwannomas are encapsulated. Most tumors can be removed with a low rate of complications and recurrence. Radiotherapy should be considered for growing lesions that are not amenable to surgery. In asymptomatic patients, observation and serial scans is an option for elderly infirm patients.