Open AccessSelinexor, a First in Class, Nuclear Export Inhibitor for the Treatment of Advanced Malignant Peripheral Nerve Sheath Tumor
Author(s) -
AlEzzi Esmail,
Gounder Mrinal,
Watson Geoffrey,
Mazzocca Alessandro,
D'Angelo Sandra P.,
Bravetti Julie,
Wang Hongwei,
Abdul Razak Albiruni,
Vincenzi Bruno
Publication year - 2021
Publication title -
the oncologist
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.176
H-Index - 164
eISSN - 1549-490X
pISSN - 1083-7159
DOI - 10.1002/onco.13692
Subject(s) - medicine , malignant peripheral nerve sheath tumor , peripheral , peripheral nerve , nuclear export signal , cancer research , surgery , pathology , anatomy , cell nucleus , immunohistochemistry , nucleus , psychiatry
Abstract 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.