Malignant Peripheral Nerve Sheath Tumor (MPNST)
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Malignant spindle cell neoplasm often arising from or in association with a peripheral nerve, from a preexisting benign nerve sheath tumor, or in a patient with neurofibromatosis type 1 (NF1). Schwann cell differentiation can sometimes still be demonstrated either histologically or immunohistochemically. Of all MPNSTs, 27–64% occur in individuals with neurofibromatosis, <10% is associated with previous irradiation, and 45–65% occur sporadic (Miao et al. 2019; Le Guellec et al. 2016). The latter group is most difficult to diagnose.
MPNST represents ~5% of the soft tissue sarcomas. MPNST may occur in patients with neurofibromatosis, an autosomal dominant tumor susceptibility syndrome affecting ~1 in 3000 newborn infants. The risk of developing MPNST in a background of NF1 is estimated at 10–15%.
References and Further Reading
- Gaspard, M., Lamant, L., Tournier, E., et al. (2018). Evaluation of eight melanocytic and neural crest-associated markers in a well-characterised series of 124 malignant peripheral nerve sheath tumours (MPNST): Useful to distinguish MPNST from melanoma? Histopathology, 73(6), 969–982.CrossRefGoogle Scholar
- Le Guellec, S., Decouvelaere, A. V., Filleron, T., et al. (2016). Malignant peripheral nerve sheath tumor is a challenging diagnosis: A systematic pathology review, immunohistochemistry, and molecular analysis in 160 patients from the French sarcoma group database. The American Journal of Surgical Pathology, 40(7), 896–908.CrossRefGoogle Scholar
- Miettinen, M. M., Antonescu, C. R., Fletcher, C. D. M., et al. (2017). Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview. Human Pathology, 67, 1–10.CrossRefGoogle Scholar