The role of miRNA deregulation in ACC was first suggested in 2007, when it was discovered that a long noncoding RNA H19 gene transcript [12] was detected in the 11p15 locus, where IGF2 is also located and associated with Beckwith–Wiedemann syndrome, which leads to the development of pediatric ACC [13]. Here, IGF2 is linked to adrenal cortex carcinoma.