Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) (previously called neurogenic sarcomas, malignant schwannomas, or neurofibrosarcomas) are soft tissue sarcomas, which arise from a peripheral nerve or show nerve sheath differentiation. MPNSTs are associated with a high risk of local recurrence and predominantly hematogenous metastasis. They account for 10 % of all soft tissue sarcomas, and approximately half of these malignancies arise in patients with neurofibromatosis type 1 (NF1). MPNSTs occur in about 2–5 % of patients with NF1 compared with an incidence of 0.001 % in the general population. In contrast, in a large population-based longitudinal study the lifetime risk of developing an MPNST in NF1 was 8–13 %. In patients with NF1, the majority of MPNSTs arise in a previous clinically detectable plexiform neurofibroma, but MPNST may also develop as a primary tumor.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Res. 2002;62(5):1573–7.
Woodruff JM. Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet. 1999;89(1):23–30.
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.
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):311–4.
King AA, Debaun MR, Riccardi VM, Gutmann DH. Malignant peripheral nerve sheath tumors in neurofibromatosis 1. Am J Med Genet. 2000;93(5):388–92.
Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH. Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions. Am J Surg Pathol. 2003;27(10):1337–45.
Fletcher CDM, Bridge JA, Hogendoorn PCW, editors. WHO classification of tumours of soft tissue and bone. Lyon: International Agency for Research on Cancer; 2013.
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.
Stein-Wexler R. Pediatric soft tissue sarcomas. Semin Ultrasound CT MR. 2011;32(5):470–88.
Evans DG, Birch JM, Ramsden RT, Sharif S, Baser ME. Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes. J Med Genet. 2006;43(4):289–94.
Guo A, Liu A, Wei L, Song X. Malignant peripheral nerve sheath tumors: differentiation patterns and immunohistochemical features—a mini-review and our new findings. J Cancer. 2012;3:303–9.
Bernthal NM, Jones KB, Monument MJ, Liu T, Viskochil D, Randall RL. Lost in translation: ambiguity in nerve sheath tumor nomenclature and its resultant treatment effect. Cancer. 2013;5(2):519–28.
Costa J, Wesley RA, Glatstein E, Rosenberg SA. The grading of soft tissue sarcomas. Results of a clinicohistopathologic correlation in a series of 163 cases. Cancer. 1984;53(3):530–41.
Trojani M, Contesso G, Coindre JM, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33(1):37–42.
Ferner RE, Golding JF, Smith M, et al. [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 2008;19(2):390–4.
Nielsen GP, Stemmer-Rachamimov AO, Ino Y, Moller MB, Rosenberg AE, Louis DN. Malignant transformation of neurofibromas in neurofibromatosis 1 is associated with CDKN2A/p16 inactivation. Am J Pathol. 1999;155(6):1879–84.
Beert E, Brems H, Daniels B, et al. Atypical neurofibromas in neurofibromatosis type 1 are premalignant tumors. Genes Chromosomes Cancer. 2011;50(12):1021–32.
Brekke HR, Kolberg M, Skotheim RI, et al. Identification of p53 as a strong predictor of survival for patients with malignant peripheral nerve sheath tumors. Neuro Oncol. 2009;11(5):514–28.
Li H, Velasco-Miguel S, Vass WC, Parada LF, DeClue JE. Epidermal growth factor receptor signaling pathways are associated with tumorigenesis in the Nf1:p53 mouse tumor model. Cancer Res. 2002;62(15):4507–13.
Mertens F, Dal Cin P, De Wever I, et al. Cytogenetic characterization of peripheral nerve sheath tumours: a report of the CHAMP study group. J Pathol. 2000;190(1):31–8.
Hollmann TJ, Hornick JL. INI1-deficient tumors: diagnostic features and molecular genetics. Am J Surg Pathol. 2011;35(10):e47–63.
Rodriguez FJ, Stratakis CA, Evans DG. Genetic predisposition to peripheral nerve neoplasia: diagnostic criteria and pathogenesis of neurofibromatoses, Carney complex, and related syndromes. Acta Neuropathol. 2012;123(3):349–67.
Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004;54(2):94–109.
Kolberg M, Holand M, Agesen TH, et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 2013;15(2):135–47.
Messiaen LM, Callens T, Mortier G, et al. Exhaustive mutation analysis of the NF1 gene allows identification of 95% of mutations and reveals a high frequency of unusual splicing defects. Hum Mutat. 2000;15(6):541–55.
Bottillo I, Ahlquist T, Brekke H, et al. Germline and somatic NF1 mutations in sporadic and NF1-associated malignant peripheral nerve sheath tumours. J Pathol. 2009;217(5):693–701.
Wimmer K, Yao S, Claes K, et al. Spectrum of single- and multiexon NF1 copy number changes in a cohort of 1,100 unselected NF1 patients. Genes Chromosomes Cancer. 2006;45(3):265–76.
Brekke HR, Ribeiro FR, Kolberg M, et al. Genomic changes in chromosomes 10, 16, and X in malignant peripheral nerve sheath tumors identify a high-risk patient group. J Clin Oncol. 2010;28(9):1573–82.
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.
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.
Perrone F, Da Riva L, Orsenigo M, et al. PDGFRA, PDGFRB, EGFR, and downstream signaling activation in malignant peripheral nerve sheath tumor. Neuro Oncol. 2009;11(6):725–36.
Kourea HP, Orlow I, Scheithauer BW, Cordon-Cardo C, Woodruff JM. Deletions of the INK4A gene occur in malignant peripheral nerve sheath tumors but not in neurofibromas. Am J Pathol. 1999;155(6):1855–60.
Joseph NM, Mosher JT, Buchstaller J, et al. The loss of Nf1 transiently promotes self-renewal but not tumorigenesis by neural crest stem cells. Cancer Cell. 2008;13(2):129–40.
Subramanian S, Thayanithy V, West RB, et al. Genome-wide transcriptome analyses reveal p53 inactivation mediated loss of miR-34a expression in malignant peripheral nerve sheath tumours. J Pathol. 2010;220(1):58–70.
Holtkamp N, Atallah I, Okuducu AF, et al. MMP-13 and p53 in the progression of malignant peripheral nerve sheath tumors. Neoplasia. 2007;9(8):671–7.
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.
Verdijk RM, den Bakker MA, Dubbink HJ, Hop WC, Dinjens WN, Kros JM. TP53 mutation analysis of malignant peripheral nerve sheath tumors. J Neuropathol Exp Neurol. 2010;69(1):16–26.
Thomas L, Mautner VF, Cooper DN, Upadhyaya M. Molecular heterogeneity in malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1. Hum Genomics. 2012;6:18.
Birindelli S, Perrone F, Oggionni M, et al. Rb and TP53 pathway alterations in sporadic and NF1-related malignant peripheral nerve sheath tumors. Lab Invest. 2001;81(6):833–44.
Legius E, Dierick H, Wu R, et al. TP53 mutations are frequent in malignant NF1 tumors. Genes Chromosomes Cancer. 1994;10(4):250–5.
Menon AG, Anderson KM, Riccardi VM, et al. Chromosome 17p deletions and p53 gene mutations associated with the formation of malignant neurofibrosarcomas in von Recklinghausen neurofibromatosis. Proc Natl Acad Sci U S A. 1990;87(14):5435–9.
Lothe RA, Smith-Sorensen B, Hektoen M, et al. Biallelic inactivation of TP53 rarely contributes to the development of malignant peripheral nerve sheath tumors. Genes Chromosomes Cancer. 2001;30(2):202–6.
Cichowski K, Shih TS, Schmitt E, et al. Mouse models of tumor development in neurofibromatosis type 1. Science. 1999;286(5447):2172–6.
Vogel KS, Klesse LJ, Velasco-Miguel S, Meyers K, Rushing EJ, Parada LF. Mouse tumor model for neurofibromatosis type 1. Science. 1999;286(5447):2176–9.
Holtkamp N, Malzer E, Zietsch J, et al. EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy. Neuro Oncol. 2008;10(6):946–57.
Bradtmoller M, Hartmann C, Zietsch J, et al. Impaired Pten expression in human malignant peripheral nerve sheath tumours. PLoS One. 2012;7(11):e47595.
Gregorian C, Nakashima J, Dry SM, et al. PTEN dosage is essential for neurofibroma development and malignant transformation. Proc Natl Acad Sci U S A. 2009;106(46):19479–84.
Keng VW, Watson AL, Rahrmann EP, et al. Conditional inactivation of Pten with EGFR overexpression in Schwann cells models sporadic MPNST. Sarcoma. 2012;2012:620834.
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.
Mawrin C, Kirches E, Boltze C, Dietzmann K, Roessner A, Schneider-Stock R. Immunohistochemical and molecular analysis of p53, RB, and PTEN in malignant peripheral nerve sheath tumors. Virchows Arch. 2002;440(6):610–5.
Wallace MR, Rasmussen SA, Lim IT, Gray BA, Zori RT, Muir D. Culture of cytogenetically abnormal schwann cells from benign and malignant NF1 tumors. Genes Chromosomes Cancer. 2000;27(2):117–23.
Upadhyaya M, Spurlock G, Thomas L, et al. Microarray-based copy number analysis of neurofibromatosis type-1 (NF1)-associated malignant peripheral nerve sheath tumors reveals a role for Rho-GTPase pathway genes in NF1 tumorigenesis. Hum Mutat. 2012;33(4):763–76.
Chai G, Liu N, Ma J, et al. MicroRNA-10b regulates tumorigenesis in neurofibromatosis type 1. Cancer Sci. 2010;101(9):1997–2004.
Perry A, Kunz SN, Fuller CE, et al. Differential NF1, p16, and EGFR patterns by interphase cytogenetics (FISH) in malignant peripheral nerve sheath tumor (MPNST) and morphologically similar spindle cell neoplasms. J Neuropathol Exp Neurol. 2002;61(8):702–9.
Keizman D, Issakov J, Meller I, et al. Expression and significance of EGFR in malignant peripheral nerve sheath tumor. J Neurooncol. 2009;94(3):383–8.
Tabone-Eglinger S, Bahleda R, Cote JF, et al. Frequent EGFR positivity and overexpression in high-grade areas of human MPNSTs. Sarcoma. 2008;2008:849156.
Byer SJ, Brossier NM, Peavler LT, et al. Malignant peripheral nerve sheath tumor invasion requires aberrantly expressed EGF receptors and is variably enhanced by multiple EGF family ligands. J Neuropathol Exp Neurol. 2013;72(3):219–33.
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.
Holtkamp N, Okuducu AF, Mucha J, et al. Mutation and expression of PDGFRA and KIT in malignant peripheral nerve sheath tumors, and its implications for imatinib sensitivity. Carcinogenesis. 2006;27(3):664–71.
Fan Q, Yang J, Wang G. Clinical and molecular prognostic predictors of malignant peripheral nerve sheath tumor. Clin Transl Oncol. 2013;16:191–9.
Torres KE, Zhu QS, Bill K, et al. Activated MET is a molecular prognosticator and potential therapeutic target for malignant peripheral nerve sheath tumors. Clin Cancer Res. 2011;17(12):3943–55.
Albritton K, Rankin C, Coffin C, et al. Phase II trial of erlotinib in metastatic or unresectable malignant peripheral nerve sheath tumor (MPNST). Journal of Clinical Oncology, 2006 ASCO Annual Meeting Proceedings (Post-Meeting Edition). Vol 24, No 18S (June 20 Supplement), 2006: 9518.
Chugh R, Wathen JK, Maki RG, et al. Phase II multicenter trial of imatinib in 10 histologic subtypes of sarcoma using a bayesian hierarchical statistical model. J Clin Oncol. 2009;27(19):3148–53.
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.
Schuetze S, Wathen S, Choy E, et al. Results of a Sarcoma Alliance for Research through Collaboration (SARC) phase II trial of dasatinib in previously treated, high-grade, advanced sarcoma. ASCO. 2010. J Clin Oncol. 2010;28:15s (suppl; abstr 10009).
Miller SJ, Jessen WJ, Mehta T, et al. Integrative genomic analyses of neurofibromatosis tumours identify SOX9 as a biomarker and survival gene. EMBO Mol Med. 2009;1(4):236–48.
Carbonnelle-Puscian A, Vidal V, Laurendeau I, et al. SOX9 expression increases with malignant potential in tumors from patients with neurofibromatosis 1 and is not correlated to desert hedgehog. Hum Pathol. 2011;42(3):434–43.
Pytel P, Karrison T, Can G, Tonsgard JH, Krausz T, Montag AG. Neoplasms with schwannian differentiation express transcription factors known to regulate normal schwann cell development. Int J Surg Pathol. 2010;18(6):449–57.
Miller SJ, Lan ZD, Hardiman A, et al. Inhibition of Eyes Absent Homolog 4 expression induces malignant peripheral nerve sheath tumor necrosis. Oncogene. 2010;29(3):368–79.
Presneau N, Eskandarpour M, Shemais T, et al. MicroRNA profiling of peripheral nerve sheath tumours identifies miR-29c as a tumour suppressor gene involved in tumour progression. Br J Cancer. 2013;108(4):964–72.
Weng Y, Chen Y, Chen J, Liu Y, Bao T. Identification of serum microRNAs in genome-wide serum microRNA expression profiles as novel noninvasive biomarkers for malignant peripheral nerve sheath tumor diagnosis. Med Oncol. 2013;30(2):531.
Kawai A, Kondo T, Suehara Y, Kikuta K, Hirohashi S. Global protein-expression analysis of bone and soft tissue sarcomas. Clin Orthop Relat Res. 2008;466(9):2099–106.
Dugu L, Hayashida S, Nakahara T, et al. Aberrant expression of tenascin-c and neuronatin in malignant peripheral nerve sheath tumors. Eur J Dermatol. 2010;20(5):580–4.
Yan X, Takahara M, Dugu L, et al. Expression of cathepsin K in neurofibromatosis 1-associated cutaneous malignant peripheral nerve sheath tumors and neurofibromas. J Dermatol Sci. 2010;58(3):227–9.
Gesundheit B, Parkin P, Greenberg M, et al. The role of angiogenesis in the transformation of plexiform neurofibroma into malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. J Pediatr Hematol Oncol. 2010;32(7):548–53.
Cabibi D, Zerilli M, Caradonna G, Schillaci L, Belmonte B, Rodolico V. Diagnostic and prognostic value of CD10 in peripheral nerve sheath tumors. Anticancer Res. 2009;29(8):3149–55.
Patel RM, Folpe AL. Immunohistochemistry for human telomerase reverse transcriptase catalytic subunit (hTERT): a study of 143 benign and malignant soft tissue and bone tumours. Pathology. 2009;41(6):527–32.
Scaife CL, Pisters PW. Combined-modality treatment of localized soft tissue sarcomas of the extremities. Surg Oncol Clin N Am. 2003;12(2):355–68.
Abbas JS, Holyoke ED, Moore R, Karakousis CP. The surgical treatment and outcome of soft-tissue sarcoma. Arch Surg. 1981;116(6):765–9.
Ingham S, Huson SM, Moran A, Wylie J, Leahy M, Evans DG. Malignant peripheral nerve sheath tumours in NF1: improved survival in women and in recent years. Eur J Cancer. 2011;47(18):2723–8.
Ren X, Wang J, Hu M, Jiang H, Yang J, Jiang Z. Clinical, radiological, and pathological features of 26 intracranial and intraspinal malignant peripheral nerve sheath tumors. J Neurosurg. 2013;119(3):695–708.
Yu J, Deshmukh H, Payton JE, et al. Array-based comparative genomic hybridization identifies CDK4 and FOXM1 alterations as independent predictors of survival in malignant peripheral nerve sheath tumor. Clin Cancer Res. 2011;17(7):1924–34.
Endo M, Yamamoto H, Setsu N, et al. Prognostic significance of AKT/mTOR and MAPK pathways and antitumor effect of mTOR inhibitor in NF1-related and sporadic malignant peripheral nerve sheath tumors. Clin Cancer Res. 2013;19(2):450–61.
Zehou O, Fabre E, Zelek L, et al. Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review. Orphanet J Rare Dis. 2013;8:127.
Amirian ES, Goodman JC, New P, Scheurer ME. Pediatric and adult malignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neurooncol. 2014;116(3):609–16.
Santoro A, Tursz T, Mouridsen H, et al. Doxorubicin versus CYVADIC versus doxorubicin plus ifosfamide in first-line treatment of advanced soft tissue sarcomas: a randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol. 1995;13(7):1537–45.
Verma S, Bramwell V. Dose-intensive chemotherapy in advanced adult soft tissue sarcoma. Expert Rev Anticancer Ther. 2002;2(2):201–15.
Fernberg JO, Wiklund T, Monge O, et al. Chemotherapy in soft tissue sarcoma. The Scandinavian Sarcoma Group experience. Acta Orthop Scand Suppl. 1999;285:62–8.
Donovan S, Shannon KM, Bollag G. GTPase activating proteins: critical regulators of intracellular signaling. Biochim Biophys Acta. 2002;1602(1):23–45.
Dodd RD, Mito JK, Eward WC, et al. NF1 deletion generates multiple subtypes of soft-tissue sarcoma that respond to MEK inhibition. Mol Cancer Ther. 2013;12:1906–17.
Jessen WJ, Miller SJ, Jousma E, et al. MEK inhibition exhibits efficacy in human and mouse neurofibromatosis tumors. J Clin Invest. 2013;123(1):340–7.
Bhola P, Banerjee S, Mukherjee J, et al. Preclinical in vivo evaluation of rapamycin in human malignant peripheral nerve sheath explant xenograft. Int J Cancer. 2010;126(2):563–71.
Johannessen CM, Johnson BW, Williams SMG, et al. TORC1 is essential for NF1-associated malignancies. Curr Biol. 2008;18(1):56–62.
Johannessen CM, Reczek EE, James MF, Brems H, Legius E, Cichowski K. The NF1 tumor suppressor critically regulates TSC2 and mTOR. Proc Natl Acad Sci U S A. 2005;102(24):8573–8.
De Raedt T, Walton Z, Yecies JL, et al. Exploiting cancer cell vulnerabilities to develop a combination therapy for ras-driven tumors. Cancer Cell. 2011;20(3):400–13.
Slomiany MG, Dai L, Bomar PA, et al. Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides. Cancer Res. 2009;69(12):4992–8.
Kohli L, Kaza N, Coric T, et al. 4-Hydroxytamoxifen induces autophagic death through K-Ras degradation. Cancer Res. 2013;73(14):4395–405.
Patel AV, Eaves D, Jessen WJ, et al. Ras-driven transcriptome analysis identifies aurora kinase A as a potential malignant peripheral nerve sheath tumor therapeutic target. Clin Cancer Res. 2012;18(18):5020–30.
Lopez G, Torres K, Liu J, et al. Autophagic survival in resistance to histone deacetylase inhibitors: novel strategies to treat malignant peripheral nerve sheath tumors. Cancer Res. 2011;71(1):185–96.
Wu J, Patmore DM, Jousma E, et al. EGFR-STAT3 signaling promotes formation of malignant peripheral nerve sheath tumors. Oncogene. 2014;33(2):173–80.
Rahrmann EP, Watson AL, Keng VW, et al. Forward genetic screen for malignant peripheral nerve sheath tumor formation identifies new genes and pathways driving tumorigenesis. Nat Genet. 2013;45(7):756–66.
Watson AL, Rahrmann EP, Moriarity BS, et al. Canonical Wnt/beta-catenin signaling drives human schwann cell transformation, progression, and tumor maintenance. Cancer Discov. 2013;3(6):674–89.
Mo W, Chen J, Patel A, et al. CXCR4/CXCL12 mediate autocrine cell-cycle progression in NF1-associated malignant peripheral nerve sheath tumors. Cell. 2013;152(5):1077–90.
Maris JM, Wiersma SR, Mahgoub N, et al. Monosomy 7 myelodysplastic syndrome and other second malignant neoplasms in children with neurofibromatosis type 1. Cancer. 1997;79:1438–46.
Ohishi J, Aoki M, Nabeshima K, et al. Imatinib mesylate inhibits cell growth of malignant peripheral nerve sheath tumors in vitro and in vivo through suppression of PDGFR-beta. BMC Cancer. 2013;13:224.
Jiang W, Schnabel C, Spyra M, et al. Efficacy and selectivity of nilotinib on NF1-associated tumors in vitro. J Neurooncol. 2014;116(2):231–6.
Sun D, Haddad R, Kraniak JM, Horne SD, Tainsky MA. RAS/MEK-independent gene expression reveals BMP2-related malignant phenotypes in the Nf1-deficient MPNST. Mol Cancer Res. 2013;11(6):616–27.
Demestre M, Terzi MY, Mautner V, Vajkoczy P, Kurtz A, Pina AL. Effects of pigment epithelium derived factor (PEDF) on malignant peripheral nerve sheath tumours (MPNSTs). J Neurooncol. 2013;115(3):391–9.
Chau V, Lim SK, Mo W, et al. Preclinical therapeutic efficacy of a novel pharmacologic inducer of apoptosis in malignant peripheral nerve sheath tumors. Cancer Res. 2014;74(2):586–97.
Kaplan HG. Vemurafenib treatment of BRAF V600E-mutated malignant peripheral nerve sheath tumor. J Natl Compr Cancer Netw. 2013;11(12):1466–70.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer Science+Business Media New York
About this chapter
Cite this chapter
Weiss, B., Sheil, A., Ratner, N. (2015). Malignant Peripheral Nerve Sheath Tumors. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_16
Download citation
DOI: https://doi.org/10.1007/978-1-4939-1830-0_16
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4939-1829-4
Online ISBN: 978-1-4939-1830-0
eBook Packages: MedicineMedicine (R0)