PRKAR1A and primary pigmented nodular adrenocortical disease: In a second study that included tissue from nine subjects with PPNAD (with eight of the nine harboring PRKAR1A defects), including five with macronodules, activating somatic CTNNB1 defects were identified in two of the five macronodules but not in the micronodules or in the contralateral adrenal gland, whereas all PPNAD tissues had β-catenin accumulation including within the macronodules, micronodules, and internodular tissue (79).