Although we were not able to identify a novel susceptibility variant for ACC in this study, we provide evidence for new avenues of investigation, such as molecular pathways involving HERC2, CACNA1A, KCNH3, and more importantly DCLK2. Indeed, the four probands, although they exhibit similar clinical characteristics, do not share a common genetic disorder. This evidence concerns the gene HERC2 and adrenal cortex carcinoma.