Preclinical studies demonstrated that the MEK inhibitor mirdametinib reduced proliferation in both neurofibromas and MPNSTs in vivo mouse models (neurofibromas: Nf1fl/fl; Dhh-Cre model, MPNSTs: xenograft model), prolonged survival in mice engrafted with human MPNST cells, and led to tumor shrinkage in neurofibroma mouse models [92]. This evidence concerns the gene DHH and plexiform neurofibroma.