Surgical Management of Peripheral Nerve Sheath Tumors

Peripheral nerve sheath tumors (PNSTs) represent a diverse group of neoplasms that arise from the nerve sheath and may occur sporadically or in the context of genetic syndromes like neurofibromatosis type 1 (NF1). The management of PNSTs associated with NF1, specifically neurofibromas, provides valuable insights that can be applied to the overall management of all PNSTs. Neurofibromas, benign but sometimes debilitating tumors, present unique challenges due to their involvement in crucial neural structures. In this review, we explore the indications for surgery, the principles of resection, as well as the pre-, intra-, and postoperative considerations, focusing on the critical role of neuromonitoring and tumor mapping in preserving functioning nerve roots. Additionally, we underline the importance of regular surveillance imaging in NF1, which can facilitate the early identification of lesions likely to undergo malignant transformation. A retrospective review of a decade’s worth of surgical outcomes at the National Institutes of Health is presented, demonstrating the effectiveness of these techniques in safely removing neurofibromas while minimizing nerve injury. Our analysis underscores the broader implications of these strategies for the management of all PNSTs, pointing toward a future where improved surgical techniques and medical therapy offer enhanced outcomes and quality of life for patients.