Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients with Neurofibromatosis Type 1. They are associated with poor prognosis and are the leading cause of mortality in NF1 patients. MPNSTs can also develop sporadically or following exposure to radiation. There is currently no effective targeted therapy to treat MPNSTs and surgical removal remains the mainstay treatment. Unfortunately, surgery is not always possible due to the size and location of the tumor, thus, a better understanding of MPNST initiation and development is required to design novel therapeutics. Here, we provide an overview of MPNST biology and genetics, discuss findings regarding the developmental origin of MPNST, and summarize the various model systems employed to study MPNST. Finally, we discuss current management strategies for MPNST, as well as recent developments in translating basic research findings into potential therapies.
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Acknowledgements
LQL held a Career Award for Medical Scientists from the Burroughs Wellcome Fund and is the Thomas L. Shield, M.D. endowed Professor in Dermatology. He is supported by funding from the US Department of Defense (NF210010), the National Cancer Institute of the NIH (R01 CA166593), and the Developmental and Hyperactive RAS Tumor SPORE (U54 CA196519); the Neurofibromatosis Therapeutic Acceleration Program; the NF1 Research Consortium Fund; and the Giorgio Foundation.
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Somatilaka, B.N., Sadek, A., McKay, R.M. et al. Malignant peripheral nerve sheath tumor: models, biology, and translation. Oncogene 41, 2405–2421 (2022). https://doi.org/10.1038/s41388-022-02290-1
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DOI: https://doi.org/10.1038/s41388-022-02290-1
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