Genotypically, the aggressive form of ACC is enriched for NOTCH‐activating mutations, particularly in NOTCH1 which are present in approximately 25% of cases and are significantly associated with shorter OS, higher likelihood of solid histology, higher rate of bone and liver metastases, and shorter relapse‐free survival [9, 10, 11]. This evidence concerns the gene NOTCH1 and adrenal cortex carcinoma.