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  • Extremely aggressive malignancies that generally occur in infants and young children. The most common locations are the kidney and central nervous system (CNS), although they can also arise in most soft tissue sites. Approximately two-thirds of patients will present with advanced-stage disease. Rhabdoid tumors of the kidney tend to metastasize to the CNS and lungs. Rhabdoid tumors are associated with mutations of SMARCB1 (INI-1), a tumor suppressor gene.
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