A study from 2021 that included 634 individuals with LS from 220 families (all were carrying an MSH2 germline mutation) registered between 1999 and 2018 showed that the prevalence of ACC was 0.47% (3 cases), all with the loss of expression of MSH2 and MSH6 proteins [22]. Here, MSH2 is linked to adrenal cortex carcinoma.