Among syndromes commonly associated with RMS and reported in Table 2 (24–27, 42, 43, 52–54, 64, 75, 80, 81, 86, 87, 89–92, 94, 96), a high RMS risk is associated with RASopathies-like type I neurofibromatosis (NF1) (deletions in NF1), Costello syndrome (HRAS mutations), and Noonan syndrome (germline variants activating RAS-MAPK pathway), highlighting the tight dependence of RMS on the RAS pathway, which results to be activated in 40% of sporadic ERMS (263, 266, 267). This evidence concerns the gene NF1 and embryonal rhabdomyosarcoma.