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Malignant Peripheral Nerve Sheath Tumor

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  • First Online:
Pathology of the Pleura and Mediastinum

Part of the book series: Encyclopedia of Pathology ((EP))

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Synonyms

MPNST; Malignant schwannoma; Neurofibrosarcoma

Definition

Malignant peripheral nerve sheath tumors (MPNST) are tumors arising from the peripheral nerves with differentiation toward nerve sheath components. MPNST are often associated with neurofibromatosis type 1 (NF1; von Recklinghausen disease) or can occur sporadically. In addition, they may arise de novo or in preexisting benign peripheral nerve sheath tumors. The cell of origin has been postulated to be a neural crest cell. The tumors predominantly occur in the major nerve trunks of proximal extremities, the trunk, head, and neck region of adults or rarely children. In addition to NF1, the tumors may also be associated with previous radiation exposure. The tumors can show a wide morphologic spectrum and lack specific immunohistochemical or molecular characteristics. In addition, MPNST can contain areas of divergent differentiation including mesenchymal (rhabdomyosarcoma, osteosarcoma, chondrosarcoma, angiosarcoma) or...

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References and Further Reading

  • Ducatman, B. S., Scheithauer, B. W., Piepgras, D. G., Reiman, H. M., & Ilstrup, D. M. (1986). Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer, 57, 2006–2021.

    Article  PubMed  CAS  Google Scholar 

  • Koezuka, S., Hata, Y., Sato, F., Otsuka, H., Makino, T., Tochigi, N., & Iyoda, A. (2014). Malignant peripheral nerve sheath tumor in the anterior mediastinum: A case report. Molecular Clinical Oncology, 2, 987–990.

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  • Kolarov, V., Stanić, J., Eri, Z., Zvezdin, B., Kojičić, M., & Hromis, S. (2010). Intrathoracic malignant peripheral nerve sheath tumor with poor outcome: A case report. Bosnian Journal of Basic Medical Sciences, 10, 328–330.

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  • Pekmezci M., Reuss D.E., Hirbe, A. C., Dahiya, S., Gutmann D.H., von Deimling, A., Horvai, A.E., & Perry, A. (2014). Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas. Mod Pathol. 28, 187–200.

    Article  PubMed  CAS  Google Scholar 

  • Thway, K., & Fisher, C. (2014). Malignant peripheral nerve sheath tumor: Pathology and genetics. Annals of Diagnostic Pathology, 18, 109–116.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. MD Anderson Cancer Center, Houston, TX, 77030, USA

    Annikka Weissferdt & Cesar A. Moran

Authors
  1. Annikka Weissferdt
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  2. Cesar A. Moran
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Corresponding author

Correspondence to Annikka Weissferdt .

Editor information

Editors and Affiliations

  1. Department of Pathology, The University of Mississippi Medical School, The University of Mississippi Medical Center, Jackson, MS, USA

    Timothy Craig Allen

  2. Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA

    Saul Suster

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© 2018 Springer International Publishing AG, part of Springer Nature

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Weissferdt, A., Moran, C.A. (2018). Malignant Peripheral Nerve Sheath Tumor. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_35

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  • DOI: https://doi.org/10.1007/978-3-319-66796-6_35

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-66795-9

  • Online ISBN: 978-3-319-66796-6

  • eBook Packages: MedicineReference Module Medicine

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