This special histological subtype is usually negative for estrogen receptors, progesterone receptors, and HER2 (so called “triple negative” cancers) [3]; however, recently, some authors reported a positivity for both estrogen and progesterone receptors in a case of ACC [15], as observed in the present case in which we found immunoreactivity for progesterone receptors. Here, ESR1 is linked to adrenal cortex carcinoma.