| Literature DB >> 33580196 |
Sumanth Nagabushan1,2, Loretta M S Lau1,2,3, Paulette Barahona3, Marie Wong2,3, Alexandra Sherstyuk3, Glenn M Marshall1,2,3, Vanessa Tyrrell3, Eva A Wegner4,5, Paul G Ekert2,3, Mark J Cowley2,3, Chelsea Mayoh2,3, Toby N Trahair1,2,3, Philip Crowe5,6,7, Antoinette Anazodo1,2,6, David S Ziegler8,9,10.
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.Entities:
Year: 2021 PMID： 33580196 DOI： 10.1038/s41698-021-00145-8
Source DB: PubMed Journal: NPJ Precis Oncol ISSN： 2397-768X