These findings are consistent with previous studies of chordoma patients that have reported germline defects in cancer predisposing genes (PALB2, CHEK2, ATM, NBN, BRCA2 and MLH) (Gröschel et al. 2019; Liang et al. 2018; Xia et al. 2022; Yepes et al. 2021), and support their hypothesis that genes involved in DNA repair pathways affecting homologous recombination (HR) may increase susceptibility to chordomas (Gröschel et al. 2019; Shinojima et al. 2023; Xia et al. 2022). This evidence concerns the gene CHEK2 and chordoma.