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Malignant peripheral nerve sheath tumor (MPNST) is a highly malignant neoplasm arising from peripheral nerve or its attendant sheath and is derived from Schwann or pluripotent cells of neural crest origin. Patients with recurrent, unresectable, or advanced stage disease have limited treatment options, and current therapies are associated with little benefit. In this article, we report nine cases of MPNST treated with selinexor, an orally bioavailable, selective inhibitor of nuclear export, accompanied by tumor stabilization or regression. © 2021 AlphaMed Press.


Esmail Al-Ezzi, Mrinal Gounder, Geoffrey Watson, Alessandro Mazzocca, Sandra P D'Angelo, Julie Bravetti, Hongwei Wang, Albiruni Abdul Razak, Bruno Vincenzi. Selinexor, a First in Class, Nuclear Export Inhibitor for the Treatment of Advanced Malignant Peripheral Nerve Sheath Tumor. The oncologist. 2021 Apr;26(4):e710-e714

PMID: 33512749

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