As angiogenesis appears critical to the development of resistance to treatment with sirolimus in vivo and the tolerability of sunitinib with an mTOR inhibitor raised concerns, we elected to develop a phase II trial combining the mTOR inhibitor everolimus with the recombinant humanized anti-VEGF monoclonal antibody bevacizumab to evaluate the clinical activity of this combination in patients with sporadic or NF1-associated MPNST. Here, VEGFA is linked to malignant peripheral nerve sheath tumor.