The present study demonstrated high expression of StAR in tumors with mutations in PRKACA, GNAS, and PRKAR1A. This finding may support a role for StAR in the activation of cAMP/PKA signaling, which is consistent with a previous study that showed increased staining of StAR in primary pigmented nodular adrenocortical disease (PPNAD) compared to control tissues [20]. Here, GNAS is linked to primary pigmented nodular adrenocortical disease.