Recently, near-simultaneous genomic studies demonstrated that SCCOHTs show inactivating mutations in SMARCA4, accompanied by loss of expression of its protein product BRG1, the same mutation that has been described in a proportion of atypical teratoid/rhabdoid tumors (ATRT) of the brain as well as extracranial MRT (2–4). This evidence concerns the gene SMARCA4 and atypical teratoid rhabdoid tumor.