Abstract
Primary adrenal insufficiency (AI) is characterized by insufficient adrenal cortisol production, even with very high adrenocorticotrophic hormone (ACTH) stimulation. Despite massive stimulation of the adrenal cortex in primary AI, there is scarce data describing an association between autoimmune AI and adrenal tumors. This report describes a 17-year-old female presenting with secondary amenorrhea, elevated 17-hydroxyprogesterone (17-OHP) and a lipid-poor adrenal tumor. She was subsequently found to have very low morning cortisol, markedly high ACTH, and positive anti-adrenal antibodies. The diagnosis of autoimmune adrenalitis causing primary AI was made. Fluorodeoxyglucose positron emission tomography (FDG-PET) scan demonstrated intense uptake (standardized uptake value (SUV) of 19.4) in the adrenal lesion. Laparoscopic adrenalectomy revealed a benign adrenal cortical adenoma. Her 17-OHP normalized postoperatively, and her menses resumed with treatment of adrenal insufficiency. This case highlights an interesting coincidence of an adrenal adenoma and primary autoimmune adrenalitis, resulting in elevated 17-OHP and amenorrhea. Low expression of 21-hydroxylase in adrenal adenomas has been previously documented and is suspected in this patient, unmasked by chronic ACTH stimulation.