Skip to main content
SpringerLink
Log in

Malignant Peripheral Nerve Sheath Tumor (MPNST) and Triton Tumor

  • Chapter
  • First Online:
Management of Soft Tissue Sarcoma

  • 2424 Accesses

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.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. 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.

    Article  PubMed  CAS  Google Scholar 

  2. Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009;11(4):322–8.

    Article  PubMed  Google Scholar 

  3. 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.

    Article  PubMed  Google Scholar 

  4. 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.

    Article  PubMed  CAS  Google Scholar 

  5. 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.

    Article  PubMed  CAS  Google Scholar 

  6. Woodruff JM, Chernik NL, Smith MC, et al. Peripheral nerve tumors with rhabdomyosarcomatous differentiation (malignant “Triton” tumors). Cancer. 1973;32(2):426–39.

    Article  PubMed  CAS  Google Scholar 

  7. Nielsen TO, West RB, Linn SC, et al. Molecular characterisation of soft tissue tumours: a gene expression study. Lancet. 2002;359(9314):1301–7.

    Article  PubMed  CAS  Google Scholar 

  8. 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.

    Article  PubMed  CAS  Google Scholar 

  9. 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.

    Article  PubMed  CAS  Google Scholar 

  10. 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.

    Article  PubMed  Google Scholar 

  11. Holtkamp N, Reuss DE, Atallah I, et al. Subclassification of nerve sheath tumors by gene expression profiling. Brain Pathol. 2004;14(3):258–64.

    Article  PubMed  CAS  Google Scholar 

  12. 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.

    Article  PubMed  CAS  Google Scholar 

  13. 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.

    Article  PubMed  Google Scholar 

  14. 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.

    Article  PubMed  CAS  Google Scholar 

  15. 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.

    Article  PubMed  CAS  Google Scholar 

  16. 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.

    Article  PubMed  CAS  Google Scholar 

  17. 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.

    Article  PubMed  CAS  Google Scholar 

  18. 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.

    Article  PubMed  CAS  Google Scholar 

  19. 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.

    Article  PubMed  CAS  Google Scholar 

  20. 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.

    Article  PubMed  CAS  Google Scholar 

  21. 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.

    Article  PubMed  CAS  Google Scholar 

  22. 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.

    Article  PubMed  Google Scholar 

  23. Cashen DV, Parisien RC, Raskin K, et al. Survival data for patients with malignant schwannoma. Clin Orthop Relat Res. 2004;426:69–73.

    Article  PubMed  Google Scholar 

  24. 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.

    Article  PubMed  CAS  Google Scholar 

  25. 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.

    Article  PubMed  CAS  Google Scholar 

  26. Evans DG, Baser ME, McGaughran J, et al. Malignant peripheral nerve sheath tumours in neurofibromatosis 1. J Med Genet. 2002;39(5):311–4.

    Article  PubMed  CAS  Google Scholar 

  27. 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.

    Article  PubMed  CAS  Google Scholar 

  28. Sordillo PP, Helson L, Hajdu SI, et al. Malignant schwannoma–clinical characteristics, survival, and response to therapy. Cancer. 1981;47(10):2503–9.

    Article  PubMed  CAS  Google Scholar 

  29. 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.

    Article  PubMed  CAS  Google Scholar 

  30. 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.

    Article  PubMed  Google Scholar 

  31. Gupta G, Mammis A, Maniker A. Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 2008;19(4):533–43.

    Article  PubMed  Google Scholar 

  32. 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.

    Article  PubMed  CAS  Google Scholar 

  33. 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.

    Google Scholar 

  34. 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.

    Article  PubMed  CAS  Google Scholar 

  35. 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.

    Article  PubMed  CAS  Google Scholar 

  36. 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.

    Article  PubMed  CAS  Google Scholar 

  37. 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.

    Article  PubMed  CAS  Google Scholar 

  38. 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.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. International Center & Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Murray F. Brennan

  2. Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA

    Cristina R. Antonescu

  3. Departments of Medicine Pediatrics, and Orthopaedics, Mount Sinai School of Medicine, New York, NY, USA

    Robert G. Maki

Authors
  1. Murray F. Brennan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cristina R. Antonescu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Robert G. Maki
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints 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)

Publish with us

Policies and ethics

Search

Navigation