Endocrine Abstracts

Episodic hypercortisolism has been widely described in Cushing’s disease (CD) but is a less commonly recognized presentation of ectopic ACTH syndrome (EAS). We describe 4 cases whose diagnosis and management was complicated by episodic hypercortisolism. These cases highlight diagnostic and therapeutic challenges during the differential diagnosis of CS complicated by intermittent hypercortisolism. All 3 cases of EAS required various combinations of ketoconazole, metyrapone and aminoglutethimide as primary medical therapy for control of hypercortisolism. While EAS is a less common cause of cyclic ACTH-dependent CS, it should be considered in cases with extreme hypercortisolism or hypokalemia. In cases with occult EAS, bilateral adrenalectomy (BAD) may be required due to difficulties in safely achieving long-term eucortisolism with medical therapy.