As with Notch signaling, alterations in chromatin remodeling genes are significantly more common in R/M ACC than primary tumors, including KDM6A mutations in 15% and CREBBP mutations in 11%, as well as other members of the SWI/SNF complex (i.e., the ARID family) [28,32]. Here, KDM6A is linked to adrenal cortex carcinoma.