Typical genetic alteration is the somatic mutation of SMARCB1 (formerly INI-1) or SMARCA4 (formerly BRG-1), retained immunohistochemical hallmarks of this tumor and demonstrated being an essential component of the ATP-dependent chromatin remodeling SWI/SNF complex, interacting with various pathways (p16-Rb pathway, Wnt-β-catenin pathway, sonic hedgehog signal pathway, polycomb pathway, MYCC, Aurora A) important for lineage specification, maintenance of stem cell pluripotency, and gene regulation (8). This evidence concerns the gene SMARCB1 and neoplasm.