Both downregulation of EZH2 by si/shRNA or pharmacological inhibition of EZH2 in the S462 (NF1-derived MPNST) and MPNST724 (spontaneous MPNST) cell lines severely affected cellular proliferation rates, induced apoptosis and interfered with tumor formation in an MPNST724 xenograft model. This evidence concerns the gene NF1 and malignant peripheral nerve sheath tumor.