Synonyms
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.
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.
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.
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.
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.
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.
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.
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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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