Characteristic of Aldosterone-Producing adrenocortical carcinoma: review of the literature.

Adrenocortical carcinoma (ACC) is a rare tumor, and aldosterone-producing-ACCs are even less frequent. Data on aldosterone-producing ACC (APAC) are scarce and most of the current knowledge derives from case reports. Therefore, aiming to summarize the main clinical features described in this aggressive cancer, we conducted a review of the PubMed database on this topic from 2005 to 2024. We included only studies with histological confirmation of ACC and biochemical evidence of aldosterone hypersecretion, identifying 27 reported cases of APAC. Epidemiological and radiological features of APACs resemble those of ACCs in general, whereas the median Ki67 index appears higher (25% vs. 10-14%) and the prevalence of ENSAT stage I greater (18% vs. 5-6%) compared with the overall ACC population, although a meaningful comparison would require a specifically designed prospective study. This is the first review assessing Ki67 and ENSAT stage in APACs based on previously published cases. To date, no significant differences have been described in morphological, clinical, or molecular aspects between these two types of ACC. Larger and higher-quality studies are needed to confirm these findings.