In addition to the germline PDE11A nonsense variant, the tumor acquired a pathogenic variant in GNAS. These findings agree with the role of PDE11A in genetic predisposition to the development of adrenal tumors [19,23], and that additional cooperating events leading to altered cAMP/PKA signaling [24] are required to drive adrenocortical tumorigenesis. Here, GNAS is linked to adrenal gland neoplasm.