Despite the identification of numerous genes with dysregulated expression in vitro or in vivo in chordoma (e.g. brachyury, EGFR, Ezrin, MMP-9, c-MET, FHIT, etc. [72]) conventional or targeted chemotherapeutic agents showed a partial response at best-case scenarios in clinical case-series. The gene discussed is EZR; the disease is chordoma.