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Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations
Author(s) -
Grobmyer Stephen R.,
Reith John D.,
Shahlaee Amir,
Bush Charles H.,
Hochwald Steven N.
Publication year - 2008
Publication title -
journal of surgical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.201
H-Index - 111
eISSN - 1096-9098
pISSN - 0022-4790
DOI - 10.1002/jso.20971
Subject(s) - medicine , neurofibromatosis , malignant peripheral nerve sheath tumor , pathogenesis , radiation therapy , surgical resection , neurofibromatoses , peripheral nerve , chemotherapy , peripheral , pathology , surgery , anatomy
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. J. Surg. Oncol. 2008;97:340–349. © 2008 Wiley‐Liss, Inc.