Two cases of MACS due to PBMAH associated with an in vivo aberrant response to LHRH treated with leuprolide acetate

Systematic dynamic stimulation testing to assess cortisol response to a variety of stimuli should be offered to all patients with primary bilateral adrenal hyperplasia (PBMAH) with indication for treatment. Identification of an aberrant response suggesting potential overexpression of β-adrenergic or luteinizing hormone/chorionic gonadotropin receptors (LHCGRs) may allow the use of targeted medical therapies, instead of unilateral or bilateral adrenalectomy, to control the cortisol excess in a subset of patients. In patients with MACS who have an aberrant cortisol response following LHRH stimulation, the long-acting GnRH agonist leuprolide acetate can be used to suppress endogenous LH production and improve hypercortisolism. A greater increase in cortisol secretion following LHRH stimulation and small response to the other stimuli could potentially be predictive of a better response to treatment with leuprolide acetate.