Advertisement

Malignant Peripheral Nerve Sheath Tumors

  • Yazgi Koy
  • Tarik Tihan
Chapter

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.

Keywords

Malignant neurofibroma Malignant schwannoma Peripheral nerve sheath tumor MPNST Schwann cell 

References

  1. 1.
    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.Google Scholar
  2. 2.
    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.CrossRefPubMedGoogle Scholar
  3. 3.
    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.CrossRefPubMedGoogle Scholar
  4. 4.
    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.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    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.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    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.CrossRefPubMedGoogle Scholar
  7. 7.
    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.CrossRefPubMedGoogle Scholar
  8. 8.
    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.CrossRefPubMedGoogle Scholar
  9. 9.
    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.CrossRefPubMedGoogle Scholar
  10. 10.
    Upadhyaya M. Genetic basis of tumorigenesis in NF1 malignant peripheral nerve sheath tumors. Front Biosci. 2011;16:937–51.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG 2016

Authors and Affiliations

  1. 1.Department of PathologyBagcilar Research and Training HospitalIstanbulTurkey
  2. 2.Department of PathologyNeuropathology DivisionSan FranciscoUSA

Personalised recommendations