Loss destabilizes the complex and disrupts its equilibrium with the polycomb repressive complex 2 (PRC2) resulting in dysregulation of transcription activity.11, 12 In addition to RMC, malignant rhabdoid tumors (MRTs) also are characterized by complete inactivation of the SMARCB1 gene.13 This evidence concerns the gene SMARCB1 and rhabdoid tumor.