Over the past few decades, there have been great successes in treating other types of cancer by identifying and effectively targeting a “hallmark mutation.” As discussed, the recurrent mutations in ACC, such as CTNNB1, p53, Rb, ATRX/DAXX, and TERT, provide some insight into the disease development but few are targetable. This evidence concerns the gene TP53 and adrenal cortex carcinoma.