NR0B1 and adenoma: Finally, few patients with diagnosis of PA and additional genetic syndromes have been reported in the literature, for example, a patient with Beckwith-Wiedemann syndrome (loss of methylation of imprinting critical region 2 on chromosome 11p) and ACTH-secreting adenoma, two family members with Cantu syndrome (ABCC9) and non-functioning adenomas, a patient with autosomal dominant polycystic kidney disease (PKD1) and acromegaly, and a patient with X-linked adrenal hypoplasia (due to a DAX1 defect) and ACTH-secreting adenoma [97,98,99,100].