Recent high throughput sequencing studies revealed some genomic mutations remarks that be identified as “drive events” of ACC tumorigenesis including mutations in IGF-2, TP53, CTNNB1, MEN1 and PRKAR1A. Somatic mutations and fusions of these genes mostly relate to continuous activation of WNT/β-catenin pathway which cause the overexpression of corresponding growth factors and inactivation of tumor suppressor TP53. In the meantime, there is a dearth of studies on the impacts of copy number variation (CNV) in ACC. This evidence concerns the gene IGF2 and adrenal cortex carcinoma.