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Malignant Peripheral Nerve Sheath Tumors—A Comprehensive Review of Pathophysiology, Diagnosis, and Multidisciplinary Management.
- Source :
- Children; Jan2022, Vol. 9 Issue 1, p38-38, 1p
- Publication Year :
- 2022
-
Abstract
- Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas (STS) with nerve sheath differentiation and a tendency to metastasize. Although occurring at an incidence of 0.001% in the general population, they are relatively common in individuals with neurofibromatosis type 1 (NF1), for whom the lifetime risk approaches 10%. The staging of MPNSTs is complicated and requires close multi-disciplinary collaboration. Their primary management is most often surgical in nature, with non-surgical modalities playing a supportive, necessary role, particularly in metastatic, invasive, or widespread disease. We, therefore, sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors, their pathophysiology and risk factors, their diagnosis, and their multi-disciplinary treatment. A close partnership between surgical and medical oncologists is therefore necessary. Advances in the molecular characterization of these tumors have also begun to allow the integration of targeted RAS/RAF/MEK/ERK pathway inhibitors into MPNST management. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 22279067
- Volume :
- 9
- Issue :
- 1
- Database :
- Complementary Index
- Journal :
- Children
- Publication Type :
- Academic Journal
- Accession number :
- 154804890
- Full Text :
- https://doi.org/10.3390/children9010038