SWI/SNF-deficient tumors of the central nervous system: An update

Atypical teratoid/rhabdoid tu-mor (AT/RT) is a highly malignant tumor of the central nervous system characterized by biallelic inactivation of SWI/SNF chromatin remodeling complex members SMARCB1/ INI1 or (rarely) SMARCA4/BRG1. Most high-grade central nervous system lesions show-ing loss of nuclear SMARCB1 or SMARCA4 protein expression can indeed be catego-rized as AT/RT. However, some high-grade lesions have been identified, whose clinical and/or molecular features justify separation from AT/RT. Furthermore, other recently de-scribed tumor types such as desmoplastic myxoid tumor, SMARCB1-mutant, and low-grade diffusely infiltrative tumor, SMARCB1-mutant, may even manifest as low-grade le-sions. Here, we review recent developments in the definition of the molecular landscape of AT/RT and give an update on other rare high-and low-grade SWI/SNF-deficient cen-tral nervous system tumors.