Germline mutations in phosphodiesterase isoform 11A (PDE11A) (2q31.2) (23) and isoform 8B (PDE8B) (5q13) (71) have been described in few cases of i-MAD (Table 3). Moreover, somatic beta-catenin gene (CTNNB1) mutations were found in larger adrenocortical nodules of 11% of patients with PPNAD in one series (63). Here, CTNNB1 is linked to primary pigmented nodular adrenocortical disease.