Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) (ICD-O 9540/3) are rare soft-tissue sarcomas that arise predominantly from Schwann cells. A malignant spindle-cell tumor with histological, immunohistochemical, and ultrastructural features of Schwann cell differentiation, they usually arise in the setting of neurofibromatosis 1 (NF1) or may be seen sporadically in children.
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References
Fletcher CDM, World Health Organization, International Agency for Research on Cancer. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. p. 468.
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;116:609–16.
Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS. Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. Pediatr Blood Cancer. 2014;61:1955–60.
Kim A, Gillespie A, Dombi E, Goodwin A, Goodspeed W, Fox E, et al. Characteristics of children enrolled in treatment trials for NF1-related plexiform neurofibromas. Neurology. 2009;73:1273–9.
Nguyen R, Dombi E, Widemann BC, Solomon J, Fuensterer C, Kluwe L, et al. Growth dynamics of plexiform neurofibromas: a retrospective cohort study of 201 patients with neurofibromatosis 1. Orphanet J Rare Dis. 2012;7:75.
Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 2010;194:1568–74.
Kivrak AS, Koc O, Emlik D, Kiresi D, Odev K, Kalkan E. Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: imaging features. Eur J Radiol. 2009;71:29–41.
Kang Y, Pekmezci M, Folpe AL, Ersen A, Horvai AE. Diagnostic utility of SOX10 to distinguish malignant peripheral nerve sheath tumor from synovial sarcoma, including intraneural synovial sarcoma. Mod Pathol. 2014;27:55–61.
Pekmezci M, Reuss DE, Hirbe AC, Dahiya S, Gutmann DH, von Deimling A, et al. Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas. Mod Pathol. 2015;28:187–200.
Upadhyaya M. Genetic basis of tumorigenesis in NF1 malignant peripheral nerve sheath tumors. Front Biosci. 2011;16:937–51.
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Koy, Y., Tihan, T. (2016). Malignant Peripheral Nerve Sheath Tumors. In: Adesina, A., Tihan, T., Fuller, C., Poussaint, T. (eds) Atlas of Pediatric Brain Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-33432-5_16
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DOI: https://doi.org/10.1007/978-3-319-33432-5_16
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