Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.

@article{Widemann2009CurrentSO,
  title={Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.},
  author={Brigitte C Widemann},
  journal={Current oncology reports},
  year={2009},
  volume={11 4},
  pages={322-8}
}
Malignant peripheral nerve sheath tumors (MPNSTs) are highly aggressive soft tissue sarcomas that rarely occur in the general population but have a lifetime incidence of 8% to 13% in those with neurofibromatosis type 1 (NF1). Complete surgical resection is the standard treatment for MPNSTs. Unresectable MPNSTs carry a poor prognosis, and survival appears to be worse in NF1-associated tumors than in sporadic tumors. The response rate of MPNSTs to standard chemotherapeutic agents used to treat… CONTINUE READING

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