Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are tumors that arise from cellular components of a normal nerve, i.e., Schwann cells and perineurial cells. They are relatively uncommon and very aggressive soft-tissue tumors seen in two settings, sporadic and associated with neurofibromatosis (NF) type 1. Despite the two clinical scenarios, the biological background, loss of NF1 expression by deletion or mutation, is believed to be similar. Older, abandoned terms for MPNST include neurofibrosarcoma, malignant schwannoma, and neurogenic sarcoma.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Vauthey JN, Woodruff JM, Brennan MF. Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience. Ann Surg Oncol. 1995;2(2):126–31.
Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009;11(4):322–8.
Basu S, Nair N. Potential clinical role of FDG-PET in detecting sarcomatous transformation in von Recklinghausen’s disease: a case study and review of the literature. J Neurooncol. 2006;80(1):91–5.
Leong AS, Wannakrairot P. A retrospective analysis of immunohistochemical staining in identification of poorly differentiated round cell and spindle cell tumors–results, reagents and costs. Pathology. 1992;24(4):254–60.
Lodding P, Kindblom LG, Angervall L. Metastases of malignant melanoma simulating soft tissue sarcoma A clinico-pathological, light- and electron microscopic and immunohistochemical study of 21 cases. Virchows Arch A Pathol Anat Histopathol. 1990;417(5):377–88.
Woodruff JM, Chernik NL, Smith MC, et al. Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “Triton” tumors). Cancer. 1973;32(2):426–39.
Nielsen TO, West RB, Linn SC, et al. Molecular characterisation of soft tissue tumours: a gene expression study. Lancet. 2002;359(9314):1301–7.
Segal NH, Pavlidis P, Antonescu CR, et al. Classification and subtype prediction of adult soft tissue sarcoma by functional genomics. Am J Pathol. 2003;163(2):691–700.
Miller SJ, Rangwala F, Williams J, et al. Large-scale molecular comparison of human schwann cells to malignant peripheral nerve sheath tumor cell lines and tissues. Cancer Res. 2006;66(5):2584–91.
Holtkamp N, Mautner VF, Friedrich RE, et al. Differentially expressed genes in neurofibromatosis 1-associated neurofibromas and malignant peripheral nerve sheath tumors. Acta Neuropathol. 2004;107(2):159–68.
Holtkamp N, Reuss DE, Atallah I, et al. Subclassification of nerve sheath tumors by gene expression profiling. Brain Pathol. 2004;14(3):258–64.
Levy P, Bieche I, Leroy K, et al. Molecular profiles of neurofibromatosis type 1-associated plexiform neurofibromas: identification of a gene expression signature of poor prognosis. Clin Cancer Res. 2004;10(11):3763–71.
Levy P, Vidaud D, Leroy K, et al. Molecular profiling of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1, based on large-scale real-time RT-PCR. Mol Cancer. 2004;3:20.
Watson MA, Perry A, Tihan T, et al. Gene expression profiling reveals unique molecular subtypes of Neurofibromatosis Type I-associated and sporadic malignant peripheral nerve sheath tumors. Brain Pathol. 2004;14(3):297–303.
Badache A, De Vries GH. Neurofibrosarcoma-derived Schwann cells overexpress platelet-derived growth factor (PDGF) receptors and are induced to proliferate by PDGF BB. J Cell Physiol. 1998;177(2):334–42.
Badache A, Muja N, De Vries GH. Expression of Kit in neurofibromin-deficient human Schwann cells: role in Schwann cell hyperplasia associated with type 1 neurofibromatosis. Oncogene. 1998;17(6):795–800.
Dang I, Nelson JK, DeVries GH. c-Kit receptor expression in normal human Schwann cells and Schwann cell lines derived from neurofibromatosis type 1 tumors. J Neurosci Res. 2005;82(4):465–71.
Mantripragada KK, Diaz de Stahl T, Patridge C, et al. Genome-wide high-resolution analysis of DNA copy number alterations in NF1-associated malignant peripheral nerve sheath tumors using 32K BAC array. Genes Chromosomes Cancer. 2009;48(10):897–907.
Mantripragada KK, Spurlock G, Kluwe L, et al. High-resolution DNA copy number profiling of malignant peripheral nerve sheath tumors using targeted microarray-based comparative genomic hybridization. Clin Cancer Res. 2008;14(4):1015–24.
Upadhyaya M, Han S, Consoli C, et al. Characterization of the somatic mutational spectrum of the neurofibromatosis type 1 (NF1) gene in neurofibromatosis patients with benign and malignant tumors. Hum Mutat. 2004;23(2):134–46.
Upadhyaya M, Kluwe L, Spurlock G, et al. Germline and somatic NF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). Hum Mutat. 2008;29(1):74–82.
Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral nerve sheath tumor: the Italian and German soft tissue sarcoma cooperative group. J Clin Oncol. 2005;23(33):8422–30.
Cashen DV, Parisien RC, Raskin K, et al. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res. 2004;426:69–73.
deCou JM, Rao BN, Parham DM, et al. Malignant peripheral nerve sheath tumors: the St. Jude children’s research hospital experience. Ann Surg Oncol. 1995;2(6):524–9.
Ducatman BS, Scheithauer BW, Piepgras DG, et al. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986;57(10):2006–21.
Evans DG, Baser ME, McGaughran J, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002;39(5):311–4.
Hruban RH, Shiu MH, Senie RT, et al. Malignant peripheral nerve sheath tumors of the buttock and lower extremity. A study of 43 cases. Cancer. 1990;66(6):1253–65.
Sordillo PP, Helson L, Hajdu SI, et al. Malignant schwannoma–clinical characteristics, survival, and response to therapy. Cancer. 1981;47(10):2503–9.
Wong WW, Hirose T, Scheithauer BW, et al. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys. 1998;42(2):351–60.
Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249(6):1014–22.
Gupta G, Mammis A, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 2008;19(4):533–43.
Skotheim RI, Kallioniemi A, Bjerkhagen B, et al. Topoisomerase-II alpha is upregulated in malignant peripheral nerve sheath tumors and associated with clinical outcome. J Clin Oncol. 2003;21(24):4586–91.
Albritton KH, Rankin C, Coffin CM, et al. Phase II study of erlotinib in metastatic or unresectable malignant peripheral nerve sheath tumors (MPNST). ASCO Meet Abstr. 2006;24 Suppl 18:9518.
Maki RG, D’Adamo DR, Keohan ML, et al. Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol. 2009;27(19):3133–40.
Johansson G, Mahller YY, Collins MH, et al. Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors. Mol Cancer Ther. 2008;7(5):1237–45.
Yang J, Ylipaa A, Sun Y, et al. Genomic and molecular characterization of malignant peripheral nerve sheath tumor identifies the IGF1R pathway as a primary target for treatment. Clin Cancer Res. 2011;17(24):7563–73.
Zou CY, Smith KD, Zhu QS, et al. Dual targeting of AKT and mammalian target of rapamycin: a potential therapeutic approach for malignant peripheral nerve sheath tumor. Mol Cancer Ther. 2009;8(5):1157–68.
Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): the mayo clinic experience. Ann Surg Oncol. 2012;19(3):878–85.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2013 Springer Science+Business Media New York
About this chapter
Cite this chapter
Brennan, M.F., Antonescu, C.R., Maki, R.G. (2013). Malignant Peripheral Nerve Sheath Tumor (MPNST) and Triton Tumor. In: Management of Soft Tissue Sarcoma. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5004-7_9
Download citation
DOI: https://doi.org/10.1007/978-1-4614-5004-7_9
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-5003-0
Online ISBN: 978-1-4614-5004-7
eBook Packages: MedicineMedicine (R0)