2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

Malignant Peripheral Nerve Sheath Tumor

  • Raffaele Gaeta
  • Alessandro FranchiEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4840


Malignant schwannoma; Neurofibrosarcoma


Malignant tumor derived from Schwann cells or pluripotent cells of neural crest origin.

Clinical Features

  • Incidence

    It represents 5–10% of all soft tissue sarcomas and occurs in only 0.001% of the population. Fifty percent of malignant peripheral nerve sheath tumors (MPNST) are associated with neurofibromatosis type 1 (NF1), about 10% are radiotherapy-induced (average of 17 years post-radiation), while the remainder are sporadic.

  • Age

    It generally occurs in adulthood, typically between 20 and 50 years of age.

  • Sex

    Men and women are equally affected.

  • Site

    The common sites of occurrence are limbs, trunk, head/neck, and retroperitoneum. Most MPNSTs arise in major nerve trunks (sciatic nerve, brachial and sacral plexus). Despite the rich innervations of the area, genitourinary MPNSTs are extremely rare. Single case reports of MPNST arising in the kidney, the prostate and paratesticular region, as well as in external genitalia have...

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References and Further Reading

  1. Djahangirian, O., Ngoc-Anh, T., Torno, L., & Khoury, A. (2018). Case of penile malignant peripheral nerve sheath tumor in a twenty-year-old male with neurofibromatosis type 1. International Archives of Urology and Complications, 4, 044.Google Scholar
  2. Eltoum, I. A., Moore, R. J., III, Cook, W., et al. (1999). Epithelioid variant of malignant peripheral nerve sheath tumor (malignant schwannoma) of the urinary bladder. Annals of Diagnostic Pathology, 3, 304–308.PubMedCrossRefGoogle Scholar
  3. Hsieh, K. L., Lu, C. C., Li, C. F., Feng, Y. H., & Liao, A. C. (2016). Malignant peripheral nerve sheath tumor of prostate: A rare case report and literature review. Case Reports in Urology, 2016, 9317567.PubMedPubMedCentralCrossRefGoogle Scholar
  4. Mortell, A., Amjad, B., Breatnach, F., Devaney, D., & Puri, P. (2007). Penile malignant peripheral nerve sheath tumour (schwannoma) in a three-year-old child without evidence of neurofibromatosis. European Journal of Pediatric Surgery, 17(6), 428–430.PubMedCrossRefGoogle Scholar
  5. Parekh, N., Cockrell, E., & McMahon, D. (2013). Malignant peripheral nerve sheath tumor of the penis: A case report and review of the literature. Urology, 81(5), 1067–1068.PubMedCrossRefGoogle Scholar
  6. Rober, P. E., Smith, J. B., Sakr, W., et al. (1991). Malignant peripheral nerve sheath tumor (malignant schwannoma) of urinary bladder in von Recklinghausen neurofibromatosis. Urology, 38, 473–476.PubMedCrossRefGoogle Scholar
  7. Voznesensky, M. A., Yamase, H., & Taylor, J. A., 3rd. (2009). Malignant peripheral nerve sheath tumor of the renal pelvis. Urologia Internationalis, 83(3), 370–372.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Translational Research and of New Technologies in Medicine and SurgeryUniversity of PisaPisaItaly