Thus atypical teratoid/rhabdoid tumors (central nervous system rhabdoid tumors) and renal and extrarenal rhabdoid tumors seem to be genetically related and SMARCB1 loss detection can thus play an integral part in differentiating rhabdoid tumors from other tumors with similar histologic features [33]. Here, SMARCB1 is linked to extrarenal rhabdoid tumor.