Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
Malignant peripheral nerve sheath tumors (MPNSTs) occur sporadically, after prior radiation therapy (RT), or in association with neurofibromatosis type 1 (NF1). It is controversial whether patients with NF1-associated MPNST have worse outcomes. We investigated the prognostic significance of sporadic, NF1-associated, and RT-induced MPNST.
Patients with primary high-grade MPNST from 1982 to 2011 were identified from a prospectively maintained database. Patients with sporadic MPNST were included only if the MPNST was not associated with NF1 or a neurofibroma or if it was immunohistochemically S100-positive.
We studied 105 patients; 42 had NF1-associated tumors, 49 sporadic, and 14 RT-induced. Median age at diagnosis was 38 years. Median follow-up for surviving patients was 4 years. Mean tumor diameter was 5.5 cm for RT-induced tumors and 9.7 cm for NF1-associated and sporadic tumors (P = 0.004). In multivariate analysis, factors associated with worse disease-specific survival (DSS) were larger size (HR 1.08; 95 % CI 1.04–1.13; P < 0.001) and positive margin (HR 3.30; 95 % CI 1.74–6.28; P < 0.001). Age, gender, site of disease, and S100 staining were not associated with DSS. The 3-year and median DSS were similar for NF1 and sporadic cases; combined 3-year DSS was 64 % and median DSS was 8.0 years. For RT-induced tumors, 3-year DSS was 49 % and median DSS was 2.4 years. The relationship between RT association and DSS approached statistical significance (HR 2.29; 95 % CI 0.93–5.67; P = 0.072).
- Lewis JJ, Leung D, Woodruff JM, Brennan MF. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998;228:355–65. CrossRef
- Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF. Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol. 1996;14:1679–89.
- Stucky CC, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, et al. Malignant peripheral nerve sheath tumors (MPNST): the Mayo Clinic experience. Ann Surg Oncol. 2012;19:878–85. CrossRef
- Zou C, Smith KD, Liu J, Lahat G, Myers S, Wang WL, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249:1014–22. CrossRef
- Grobmyer SR, Reith JD, Shahlaee A, Bush CH, Hochwald SN. Malignant Peripheral Nerve Sheath Tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 2008;97:340–9. CrossRef
- Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002;39:311–4. CrossRef
- Huson SM, Compston DA, Clark P, Harper PS. A genetic study of von Recklinghausen neurofibromatosis in south east Wales. I. Prevalence, fitness, mutation rate, and effect of parental transmission on severity. J Med Genet. 1989;26:704–11. CrossRef
- Sorensen SA, Mulvihill JJ, Nielsen A. Long-term follow-up of von Recklinghausen neurofibromatosis. Survival and malignant neoplasms. N Engl J Med. 1986;314:1010–5. CrossRef
- D’Agostino AN, Soule EH, Miller RH. Primary malignant neoplasms of nerves (malignant neurilemomas) in patients without manifestations of multiple neurofibromatosis (Von Recklinghausen’s disease). Cancer. 1963;16:1003–14. CrossRef
- Anghileri M, Miceli R, Fiore M, Mariani L, Ferrari A, Mussi C, et al. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006;107:1065–74. CrossRef
- Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol. 2005;23:8422–30. CrossRef
- Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986;57:2006–21. CrossRef
- Sordillo PP, Helson L, Hajdu SI, Magill GB, Kosloff C, Golbey RB, et al. Malignant schwannoma–clinical characteristics, survival, and response to therapy. Cancer. 1981;47:2503–9. CrossRef
- Porter DE, Prasad V, Foster L, Dall GF, Birch R, Grimer RJ. Survival in malignant peripheral nerve sheath tumours: a comparison between sporadic and neurofibromatosis type 1-associated tumours. Sarcoma. 2009;2009:756395. CrossRef
- Cashen DV, Parisien RC, Raskin K, Hornicek FJ, Gebhardt MC, Mankin HJ. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res. 2004:69–73.
- deCou JM, Rao BN, Parham DM, Lobe TE, Bowman L, Pappo AS, et al. Malignant peripheral nerve sheath tumors: the St. Jude Children’s Research Hospital experience. Ann Surg Oncol. 1995;2:524–9.
- Hruban RH, Shiu MH, Senie RT, Woodruff JM. Malignant peripheral nerve sheath tumors of the buttock and lower extremity. A study of 43 cases. Cancer. 1990;66:1253–65. CrossRef
- 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;42:351–60. CrossRef
- Riad S, Biau D, Holt GE, Werier J, Turcotte RE, Ferguson PC, et al. The clinical and functional outcome for patients with radiation-induced soft tissue sarcoma. Cancer. 2012;118:2682–92. CrossRef
- Mavrogenis AF, Pala E, Guerra G, Ruggieri P. Post-radiation sarcomas. Clinical outcome of 52 Patients. J Surg Oncol. 2012;105:570–6. CrossRef
- Gladdy RA, Qin LX, Moraco N, Edgar MA, Antonescu CR, Alektiar KM, et al. Do radiation-associated soft tissue sarcomas have the same prognosis as sporadic soft tissue sarcomas? J Clin Oncol. 2010;28:2064–9. CrossRef
- National Institutes of Health Consensus Development Conference Statement: neurofibromatosis. Bethesda, USA, July 13–15, 1987. Neurofibromatosis. 1988;1:172–8.
- Singer S, Maki RG, O’Sullivan B. Soft tissue sarcoma. In: DeVita VTJ, Lawrence TS, Rosenberg SA, et al., eds. Cancer: principles and practice of oncology, 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2011.
- Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors
Annals of Surgical Oncology
Volume 20, Issue 1 , pp 66-72
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- Industry Sectors
- Author Affiliations
- 1. Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
- 2. Department of Epidemiology and Biostatistics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
- 3. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA