The role of SMARCB1/INI1 in the development of rhabdoid tumors

Abstract

Rhabdoid tumors are especially lethal cancers that predominantly strike young children. The vast majority of rhabdoid tumors contain bi-allelic inactivating mutations in the SMARCB1 gene. Here we review clinicopathologic features of rhabdoid tumors and present recent insights into the mechanisms that drive oncogenesis in the absence of SMARCB1 function.