Abstract
Atypical teratoid/rhabdoid tumors (AT/RT) of the brain and malignant rhabdoid tumors of the kidney (RTK) compose a group of highly malignant pediatric cancers known as malignant rhabdoid tumors (MRT). Both subtypes are characterized by the biallelic loss of the SMARCB1 gene (member of the human SWI/SNF complex), which is thought to function as a tumor suppressor gene, and by the complete absence of its product expression INI1 protein. The occurrence of MRT is limited to patients younger than 5 years, with more than 80% of children being under 2 years old.
