SMARCB1 and neoplasm: The first genetic evidence of the role of SMARCB1 as a tumor suppressor was the identification of its biallelic mutations as the cause of most cases of malignant rhabdoid tumors (RTs)3, 4, a highly aggressive pediatric cancer that usually occurs in the brain (named atypical teratoid rhabdoid tumor: AT/RT), kidneys and soft tissues in the first years of life.