Human Pluripotent Stem Cell-Derived Tumor Model Uncovers the Embryonic Stem Cell Signature as a Key Driver in Atypical Teratoid/Rhabdoid Tumor (Cell Reports)

Terada et al. present SMARCB1-deficient human pluripotent stem cell-derived atypical teratoid/rhabdoid tumor (AT/RT) models and show that ESC-like signature is a critical driver of malignant phenotypes of AT/RT. Genetic ablation targeting the maintenance of pluripotency inhibits AT/RT cell growth, suggesting that the ESC-like signature could be a promising therapeutic target for AT/RT.