Due to the importance of immune cells in the progression of neurofibromas [24,25] and the in vivo efficacy of MSU-42011 in Kras-driven lung cancer (Figure 1) [29,30], which shares similarities with NF1 models, immunocompetent C57BL/6 mice were used to evaluate the immunomodulatory and anti-tumor effects of MSU-42011 in vivo in MPNST. This evidence concerns the gene NF1 and malignant peripheral nerve sheath tumor.