Rhabdoid tumors are a group of highly aggressive childhood cancers. They commonly occur in the brain and are known as atypical teratoid rhabdoid tumors (ATRT). Another common place they occur is the kidney, where they are referred to as malignant rhabdoid tumors of the kidney. However, rhabdoid tumors can occur anywhere in the body. They are quite rare and usually found before age II. Nearly all rhabdoid tumors are caused by a mutation in a gene called SMARCB1 (also called INI1). At MSK Kids, we use high-dose chemotherapy to treat rhabdoid tumors, which we believe provides the best chance of a cure. Our unique treatment approach is based on our experience treating children with other aggressive solid tumors. In addition, we research the cause of rhabdoid tumors. We have found that a gene called PGBD5 may contribute to these cancers developing. We are studying the role of the drug tazemetostat (TAZVERIK) to treat children with rhabdoid tumors if their first treatment doesn’t work.