The Importance of Predicting Factors in the Surgical Outcome of Peripheral Nerve Sheath Tumors

Abstract

Objective Peripheral nerve sheath tumors (PNSTs) are tumors arising from the neural sheath cells. Surgery plays a central role in the management of this disease, with the purpose of obtaining radical tumor's resection and at the same time providing the best outcome. We retrospectively analyzed 53 PNSTs in 42 patients in an attempt to identify some factors that may improve surgical outcome.

Material and Methods Clinical, histologic, and imaging data of 42 patients with PNSTs treated at our Institute between 2001 and 2012 were collected and analyzed. We evaluated the outcome 1 month and 6 month after surgery using three clinical parameters (pain, motor deficits, and sensory deficits) in relation to different histotypes, the presence of neurofibromatosis type 1, tumor location, and duration of symptoms before treatment.

Results The best functional results were observed in patients having neurofibromas; the worst outcomes were observed in patients with malignant PNSTs. The other factors were not associated with outcome.

Conclusion The timing of surgery is the most important predictive factor of surgical outcome, being the only factor that allows to improve the outcome. With the current study, we want to stress the importance of treating PNSTs as soon as possible to provide the best outcome possible.

Keywords

neurofibroma - neurofibromatosis - peripheral nerve sheath tumors - schwannoma - PNST

• References

• 1 Gupta G, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Focus 2007; 22 (6) E12
• 2 Baehring JM, Betensky RA, Batchelor TT. Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology 2003; 61 (5) 696-698
• 3 Rodriguez FJ, Folpe AL, Giannini C, Perry A. Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 2012; 123 (3) 295-319
• 4 Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG. A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. J Neurosurg 2005; 102 (2) 246-255
• 5 Stucky CCH, Johnson KN, Gray RJ , et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol 2012; 19 (3) 878-885
• 6 Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer 1986; 57 (10) 2006-2021
• 7 Hajdu SI. Peripheral nerve sheath tumors. Histogenesis, classification, and prognosis. Cancer 1993; 72 (12) 3549-3552
• 8 Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res 2002; 62 (5) 1573-1577
• 9 Murovic JA, Kim DH, Kline DG. Neurofibromatosis-associated nerve sheath tumors. Case report and review of the literature. Neurosurg Focus 2006; 20 (1) E1
• 10 Wong WW, Hirose T, Scheithauer BW, Schild SE, Gunderson LL. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998; 42 (2) 351-360
• 11 Patel NB, Stacy GS. Musculoskeletal manifestations of neurofibromatosis type I. AJR 2012; 199: W99-W106
• 12 Ferner RE, O'Doherty MJ. Neurofibroma and schwannoma. Curr Opin Neurol 2002; 15 (6) 679-684
• 13 Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet 2002; 39 (5) 311-314
• 14 Cashen DV, Parisien RC, Raskin K, Hornicek FJ, Gebhardt MC, Mankin HJ. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res 2004; 426 (426) 69-73
• 15 Kar M, Deo SV, Shukla NK , et al. Malignant peripheral nerve sheath tumors (MPNST)—clinicopathological study and treatment outcome of twenty-four cases. World J Surg Oncol 2006; 4: 55
• 16 Gachiani J, Kim D, Nelson A, Kline D. Surgical management of malignant peripheral nerve sheath tumors. Neurosurg Focus 2007; 22 (6) E13
• 17 Levi AD, Ross AL, Cuartas E, Qadir R, Temple HT. The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery 2010; 66 (4) 833-840
• 18 Wasa J, Nishida Y, Tsukushi S , et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol 2010; 194 (6) 1568-1574
• 19 Angelov L, Davis A, O'Sullivan B, Bell R, Guha A. Neurogenic sarcomas: experience at the University of Toronto. Neurosurgery 1998; 43 (1) 56-64 , discussion 64–65
• 20 LaFemina J, Qin LX, Moraco NH , et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol 2013; 20 (1) 66-72
• 21 Medical Research Council scale. Aids to examination of the peripheral nervous system. London: Her Majesty's Stationery Office 1976; Memorandum no.45.
• 22 Ferner RE, Lucas JD, O'Doherty MJ , et al. Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry 2000; 68 (3) 353-357
• 23 Benz MR, Czernin J, Dry SM , et al. Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer 2010; 116 (2) 451-458
• 24 Sunderland S. Nerves and nerve injuries. Edinburgh: Churchill Livingstone; 1978
• 25 Donner TR, Voorhies RM, Kline DG. Neural sheath tumors of major nerves. J Neurosurg 1994; 81: 62-373
• 26 Kretschmer T, Antoniadis G, Heinen C , et al. Nerve sheath tumor surgery: case-guided discussion of ambiguous findings, appropriateness of removal, repeated surgery, and nerve repairs. Neurosurg Focus 2007; 22 (6) E19
• 27 Doorn PF, Molenaar WM, Buter J, Hoekstra HJ. Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis. Eur J Surg Oncol 1995; 21 (1) 78-82
• 28 Dunn GP, Spiliopoulos K, Plotkin SR , et al. Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1. J Neurosurg 2013; 118 (1) 142-148
• 29 Anghileri M, Miceli R, Fiore M , et al. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer 2006; 107 (5) 1065-1074