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Malignant Peripheral Nerve Sheath Tumor

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

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

  • 47 Accesses

Synonyms

Malignant schwannoma; Neurofibrosarcoma

Definition

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

  • 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.

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  • 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.

    Article  CAS  Google Scholar 

  • 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.

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  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Translational Research and of New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy

    Raffaele Gaeta & Alessandro Franchi

Authors
  1. Raffaele Gaeta
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  2. Alessandro Franchi
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Corresponding author

Correspondence to Alessandro Franchi .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Gaeta, R., Franchi, A. (2019). Malignant Peripheral Nerve Sheath Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4840-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4840-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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