However monotherapy with these agents has demonstrated limited activity in models of MPNST (Wang et al. 2021), due to additional genomic alterations that drive disease progression from pNF to MPNST, such as loss of CDKN2A and PRC2 components SUZ12 or EED (Lemberg et al. 2020). This evidence concerns the gene CDKN2A and malignant peripheral nerve sheath tumor.