The OS predisposition of Li-Fraumeni patients and mouse models support the key role of p53 mutation in OS: Trp53+/- and Trp53-/- mice develop OS in addition to other tumors while conditional deletion of Trp53 in the osteoblastic lineage results in full penetrance OS, largely in the absence of other tumor types (Mutsaers and Walkley, 2014; Donehower et al., 1992; Quist et al., 2015; Wang et al., 2006; Lengner et al., 2006; Zhao et al., 2015). This evidence concerns the gene TP53 and neoplasm.