Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

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...

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Bibliographic Details
Published in:Journal of surgical oncology 2008-03, Vol.97 (4), p.340-349
Main Authors: Grobmyer, Stephen R., Reith, John D., Shahlaee, Amir, Bush, Charles H., Hochwald, Steven N.
Format: Article
Language:English
Subjects:
chemotherapy
Chemotherapy, Adjuvant
Chromosome Aberrations
Combined Modality Therapy
Humans
malignant peripheral nerve sheath tumor
Neoplasm Recurrence, Local
Nerve Sheath Neoplasms - diagnostic imaging
Nerve Sheath Neoplasms - genetics
Nerve Sheath Neoplasms - pathology
Nerve Sheath Neoplasms - therapy
neurofibromatosis
pathogenesis
Prognosis
radiation therapy
Radiography
Radiotherapy, Adjuvant
review
surgery
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Summary: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.
ISSN:0022-4790
1096-9098
DOI:10.1002/jso.20971