Document Type
Journal Article
Publication Date
1-1-2017
Journal
Sarcoma
Volume
2017
DOI
10.1155/2017/7429697
Abstract
Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and biology of MPNST through imaging and genomic advances since 2002. Genetically engineered mouse models that develop MPNST spontaneously have greatly facilitated preclinical evaluation of novel drugs for translation into clinical trials led by consortia efforts. Continued work in identifying alterations that contribute to the transformation, progression, and metastasis of MPNST coupled with longitudinal follow-up, biobanking, and data sharing is needed to develop prognostic biomarkers and effective prevention and therapeutic strategies for MPNST.
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.
APA Citation
Kim, A., Stewart, D., Reilly, K., Viskochil, D., Miettinen, M., & Widemann, B. (2017). Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies.. Sarcoma, 2017 (). http://dx.doi.org/10.1155/2017/7429697
Peer Reviewed
1
Open Access
1
Comments
Reproduced with permission of Hindawi Publishers. Sarcoma