Journal
JOURNAL OF SURGICAL ONCOLOGY
Volume 97, Issue 4, Pages 340-349Publisher
WILEY
DOI: 10.1002/jso.20971
Keywords
malignant peripheral nerve sheath tumor; neurofibromatosis; surgery; chemotherapy; radiation therapy; pathogenesis; review
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Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare tumors that often occur in patients with neurofibromatosis 1. Surgical resection represents the mainstay of treatment. Radiation and chemotherapy have a role in selected patients with MPNST. Accurate pathologic diagnosis remains a challenge in many cases of MPNST. There are many recent advances in the understanding of the molecular pathogenesis of MPNST which represent the best opportunities to develop new strategies for management of patients with MPNST.
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