In this way, the Next Sequencing Generation (NGS) genomic characterization of our models showed a preponderance (58%) of homozygous deletions of CDKN2A/2B and mutations affecting the mammalian SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complexes (such as PBRM1, SMARCB1, ARID1A pathogenic mutations), as described in a majority of chordomas (5, 6, 14). The gene discussed is PBRM1; the disease is chordoma.