Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Malignant Peripheral Nerve Sheath Tumor

  • Annikka WeissferdtEmail author
  • Cesar A. Moran
Reference work entry


MPNST; Malignant schwannoma; Neurofibrosarcoma


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

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.PubMedPubMedCentralCrossRefGoogle Scholar
  3. 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.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 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.PubMedCrossRefGoogle Scholar
  5. Thway, K., & Fisher, C. (2014). Malignant peripheral nerve sheath tumor: Pathology and genetics. Annals of Diagnostic Pathology, 18, 109–116.PubMedCrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.MD Anderson Cancer CenterHoustonUSA