Malignant Peripheral Nerve Sheath Tumor
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Malignant schwannoma; Neurofibrosarcoma
Malignant tumor derived from Schwann cells or pluripotent cells of neural crest origin.
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.
It generally occurs in adulthood, typically between 20 and 50 years of age.
Men and women are equally affected.
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...
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.Google Scholar