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

Abstract 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...

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Autores principales:	Sumanth Nagabushan, Loretta M. S. Lau, Paulette Barahona, Marie Wong, Alexandra Sherstyuk, Glenn M. Marshall, Vanessa Tyrrell, Eva A. Wegner, Paul G. Ekert, Mark J. Cowley, Chelsea Mayoh, Toby N. Trahair, Philip Crowe, Antoinette Anazodo, David S. Ziegler
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/1017be47b03249d190e20ce279c78746
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spelling	oai:doaj.org-article:1017be47b03249d190e20ce279c787462021-12-02T13:50:57ZEfficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor10.1038/s41698-021-00145-82397-768Xhttps://doaj.org/article/1017be47b03249d190e20ce279c787462021-02-01T00:00:00Zhttps://doi.org/10.1038/s41698-021-00145-8https://doaj.org/toc/2397-768XAbstract 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.Sumanth NagabushanLoretta M. S. LauPaulette BarahonaMarie WongAlexandra SherstyukGlenn M. MarshallVanessa TyrrellEva A. WegnerPaul G. EkertMark J. CowleyChelsea MayohToby N. TrahairPhilip CroweAntoinette AnazodoDavid S. ZieglerNature PortfolioarticleNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENnpj Precision Oncology, Vol 5, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Sumanth Nagabushan Loretta M. S. Lau Paulette Barahona Marie Wong Alexandra Sherstyuk Glenn M. Marshall Vanessa Tyrrell Eva A. Wegner Paul G. Ekert Mark J. Cowley Chelsea Mayoh Toby N. Trahair Philip Crowe Antoinette Anazodo David S. Ziegler Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor
description	Abstract 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.
format	article
author	Sumanth Nagabushan Loretta M. S. Lau Paulette Barahona Marie Wong Alexandra Sherstyuk Glenn M. Marshall Vanessa Tyrrell Eva A. Wegner Paul G. Ekert Mark J. Cowley Chelsea Mayoh Toby N. Trahair Philip Crowe Antoinette Anazodo David S. Ziegler
author_facet	Sumanth Nagabushan Loretta M. S. Lau Paulette Barahona Marie Wong Alexandra Sherstyuk Glenn M. Marshall Vanessa Tyrrell Eva A. Wegner Paul G. Ekert Mark J. Cowley Chelsea Mayoh Toby N. Trahair Philip Crowe Antoinette Anazodo David S. Ziegler
author_sort	Sumanth Nagabushan
title	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_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_sort	efficacy of mek inhibition in a recurrent malignant peripheral nerve sheath tumor
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/1017be47b03249d190e20ce279c78746
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Efficacy of MEK inhibition in a recurrent malignant peripheral nerve sheath tumor

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