The mechanistic target of rapamycin (mTOR) is another target with relevance to NF1 tumour biology.115 This protein has previously been studied in pNF and MPNST-focussed clinical trials, using the mTOR inhibitor sirolimus as well as the mTOR inhibitor everolimus in combination with the VEGF inhibitor bevacizumab.122,123 However, the results from these studies indicated that these interventions were inactive in pNF and MPNST, respectively. Here, NF1 is linked to malignant peripheral nerve sheath tumor.