Recent studies have described the immunohistochemical loss of SMARCB1/INI1 protein in poorly differentiated chordoma associated with SMARCB1 gene deletions at fluorescence in situ hybridization (FISH) examination, mainly deriving from large, homozygous deletions at 22q11 locus (4, 6–8). The gene discussed is SMARCB1; the disease is chordoma.