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- Title
Malignant Peripheral Nerve Sheath Tumors—A Comprehensive Review of Pathophysiology, Diagnosis, and Multidisciplinary Management.
- Authors
Knight, Samantha W. E.; Knight, Tristan E.; Santiago, Teresa; Murphy, Andrew J.; Abdelhafeez, Abdelhafeez H.
- 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.
- Subjects
SOFT tissue tumors; METASTASIS; ONCOLOGISTS; NEUROFIBROMATOSIS; ONCOLOGY; CELL differentiation; NERVOUS system tumors; NEUROFIBROMA; RISK assessment; TUMOR classification; CELLULAR signal transduction; HEALTH care teams; NEUROFIBROMATOSIS 1; DISEASE risk factors
- Publication
Children, 2022, Vol 9, Issue 1, p38
- ISSN
2227-9067
- Publication type
Article
- DOI
10.3390/children9010038