(27) described 2 cases of conventional chordomas with loss of immunohistochemical expression of SMARCB1/INI1 associated with loss of SMARCB1 locus, and Wen reported a single case of extra-axial conventional chordoma with a partial loss of SMARCB1/INI1 at immunohistochemistry despite no deletion of SMARCB1 detected by FISH analysis (9). This evidence concerns the gene SMARCB1 and chordoma.