The Surgical Management of Symptomatic Peripheral Nerve Sheath Tumors

Abstract

Abstract OBJECTIVE To determine the clinical presentation and morbidity of the surgical management of peripheral nerve sheath tumors (PNSTs). METHODS We performed a retrospective chart review of surgically treated PNSTs at the University of Miami between 1991 and 2008. RESULTS There were a total of 140 cases, including 87 schwannomas, 34 neurofibromas, and 19 malignant peripheral nerve sheath tumors (MPNSTs). The average age of the total study group was 49.0 years; it was significantly lower for patients with neurofibroma. There was a high correlation between neurofibroma tumors and neurofibromatosis-1. Most patients with benign tumors presented with a painful mass, paresthesias, or numbness without significant weakness. Patients who had previously undergone attempted resections and preoperative biopsy had a significantly increased risk (41%) for developing postoperative neurologic deficits when compared with patients who presented with de novo tumors (15%). Intraoperative monitoring appeared to reduce the risk of postoperative motor deficit, particularly in neurofibromas. Most MPNSTs (>80%) were diagnosed at stage IIB or higher and had a combined mortality rate of 31.6% at 78 months. Tumor size was the best predictor of adverse outcome, as all MPNST mortalities occurred in patients with a tumor size of more than 7 cm. CONCLUSION PNSTs are a heterogeneous group of lesions. Benign tumors respond well to marginal excision, whereas MPNSTs are aggressive sarcomas that require multimodal management. There was a significantly increased risk of postoperative neurologic deficits in patients who had undergone a previous biopsy, and thus tertiary referral without biopsy is recommended when a PNST is suspected.