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Malignant peripheral nerve sheath tumors MPNST are aggressive sarcomas characterized by high recurrence rates and early metastases. These tumors arise more frequently within neurofibromatosis type 1 NF1 and present with resistance during standard chemotherapy leading to increased mortality and morbidity in those patients. Neurofibromatosis type 1 NF1 is a risk factor for the development of malignant peripheral nerve sheath tumors MPNST , which are associated with poor prognosis due to high recurrence and early metastases [ 1 , 2 ]. Treatment involves surgical removal and chemotherapy. However, MPNST display significant resistance to standard chemotherapy [ 4 , 5 ], therefore adapted therapies are necessary. Pre-clinical transgenic mouse models demonstrated efficacy of MEKi when admitted alone, however increased MEKi properties were seen when combined with RAD [ 8 , 14 , 15 ].
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Malignant peripheral nerve sheath tumors MPNST are aggressive sarcomas characterized by high recurrence rates and early metastases. These tumors arise more frequently within neurofibromatosis type 1 NF1 and present with resistance during standard chemotherapy leading to increased mortality and morbidity in those patients. The online version of this article
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