Malignant peripheral nerve sheath tumors: an update.

  title={Malignant peripheral nerve sheath tumors: an update.},
  author={Bruno Fuchs and Robert J Spinner and Michael G. Rock},
  journal={Journal of surgical orthopaedic advances},
  volume={14 4},
Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant cancers that account for approximately 5-10% of all soft tissue sarcomas. They occur either sporadically, in association with neurofibromatosis type 1 (NF1), or subsequent to radiation therapy. Histologically, MPNSTs resemble fibrosarcomas in their basic organization. Immunohistochemistry, particularly S100, plays an important role in the diagnosis. At the molecular level, loss of the NF1 gene and high levels of Ras-activity… CONTINUE READING
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