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Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor

The prognosis of recurrent malignant peripheral nerve sheath tumors (MPNST) is dismal, with surgical resection being the only definitive salvage therapy. Treatment with chemoradiation approaches has not significantly improved patient outcomes. Similarly, trials of therapies targeting MPNST genomic d...

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Autores principales: Nagabushan, Sumanth, Lau, Loretta M. S., Barahona, Paulette, Wong, Marie, Sherstyuk, Alexandra, Marshall, Glenn M., Tyrrell, Vanessa, Wegner, Eva A., Ekert, Paul G., Cowley, Mark J., Mayoh, Chelsea, Trahair, Toby N., Crowe, Philip, Anazodo, Antoinette, Ziegler, David S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7881142/
https://www.ncbi.nlm.nih.gov/pubmed/33580196
http://dx.doi.org/10.1038/s41698-021-00145-8
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author Nagabushan, Sumanth
Lau, Loretta M. S.
Barahona, Paulette
Wong, Marie
Sherstyuk, Alexandra
Marshall, Glenn M.
Tyrrell, Vanessa
Wegner, Eva A.
Ekert, Paul G.
Cowley, Mark J.
Mayoh, Chelsea
Trahair, Toby N.
Crowe, Philip
Anazodo, Antoinette
Ziegler, David S.
author_facet Nagabushan, Sumanth
Lau, Loretta M. S.
Barahona, Paulette
Wong, Marie
Sherstyuk, Alexandra
Marshall, Glenn M.
Tyrrell, Vanessa
Wegner, Eva A.
Ekert, Paul G.
Cowley, Mark J.
Mayoh, Chelsea
Trahair, Toby N.
Crowe, Philip
Anazodo, Antoinette
Ziegler, David S.
author_sort Nagabushan, Sumanth
collection PubMed
description The prognosis of recurrent malignant peripheral nerve sheath tumors (MPNST) is dismal, with surgical resection being the only definitive salvage therapy. Treatment with chemoradiation approaches has not significantly improved patient outcomes. Similarly, trials of therapies targeting MPNST genomic drivers have thus far been unsuccessful. Improved understanding of the molecular pathogenesis of MPNST indicates frequent activation of the mitogen-activated protein kinase (MAPK) cell signaling pathway. MEK inhibitors have shown activity in preclinical studies; however, their clinical efficacy has not been reported to date. We describe here a case of sustained complete response to MEK inhibition in an adolescent patient with a recurrent metastatic MPNST with multiple alterations in the MAPK pathway, guided by a precision oncology approach.
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spelling pubmed-78811422021-02-25 Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor Nagabushan, Sumanth Lau, Loretta M. S. Barahona, Paulette Wong, Marie Sherstyuk, Alexandra Marshall, Glenn M. Tyrrell, Vanessa Wegner, Eva A. Ekert, Paul G. Cowley, Mark J. Mayoh, Chelsea Trahair, Toby N. Crowe, Philip Anazodo, Antoinette Ziegler, David S. NPJ Precis Oncol Case Report The prognosis of recurrent malignant peripheral nerve sheath tumors (MPNST) is dismal, with surgical resection being the only definitive salvage therapy. Treatment with chemoradiation approaches has not significantly improved patient outcomes. Similarly, trials of therapies targeting MPNST genomic drivers have thus far been unsuccessful. Improved understanding of the molecular pathogenesis of MPNST indicates frequent activation of the mitogen-activated protein kinase (MAPK) cell signaling pathway. MEK inhibitors have shown activity in preclinical studies; however, their clinical efficacy has not been reported to date. We describe here a case of sustained complete response to MEK inhibition in an adolescent patient with a recurrent metastatic MPNST with multiple alterations in the MAPK pathway, guided by a precision oncology approach. Nature Publishing Group UK 2021-02-12 /pmc/articles/PMC7881142/ /pubmed/33580196 http://dx.doi.org/10.1038/s41698-021-00145-8 Text en © The Author(s) 2021 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
spellingShingle Case Report
Nagabushan, Sumanth
Lau, Loretta M. S.
Barahona, Paulette
Wong, Marie
Sherstyuk, Alexandra
Marshall, Glenn M.
Tyrrell, Vanessa
Wegner, Eva A.
Ekert, Paul G.
Cowley, Mark J.
Mayoh, Chelsea
Trahair, Toby N.
Crowe, Philip
Anazodo, Antoinette
Ziegler, David S.
Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title_full Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title_fullStr Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title_full_unstemmed Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title_short Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
title_sort efficacy of mek inhibition in a recurrent malignant peripheral nerve sheath tumor
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7881142/
https://www.ncbi.nlm.nih.gov/pubmed/33580196
http://dx.doi.org/10.1038/s41698-021-00145-8
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