KIT and adrenal cortex carcinoma: Although ACC display an overall low mutation burden [11], a large subset of cases (30–80% according to multiple studies) harbor chromosomal rearrangement that produce MYB-NFIB fusion gene, which plays a central role in the ACC pathogenesis via activation of MYB target genes (such as BCL2, MYC, CD34, and KIT) that are involved in regulation of cell proliferation, differentiation, apoptosis, and angiogenesis [12–15].