Unique Sex Steroid Profiles in Estrogen-Producing Adrenocortical Adenoma Associated With Bilateral Hyperaldosteronism

Abstract Because of its rarity, our understanding of steroidogenesis in estrogen-producing adrenocortical adenoma, including the response to adrenocorticotropic hormone (ACTH) stimulation, remains limited. A 65-year-old man was referred to us because of primary aldosteronism and a right adrenal tumor. Endocrinological evaluations revealed secondary hypogonadism due to hyperestrogenemia. Adrenal venous sampling (AVS) and subsequent liquid chromatography–tandem mass spectrometry (LC-MS/MS) indicated bilateral hyperaldosteronism and a right estrogen-producing adrenocortical tumor. He subsequently underwent right unilateral adrenalectomy, which resulted in clinical remission of hypogonadism. Subsequent histopathological analysis identified a right estrogen-producing adrenocortical adenoma and multiple, concomitant adrenocortical micronodules. Sequential evaluation of steroid profiles using LC-MS/MS revealed unique hormone production, including adrenal androgens, and less responsiveness to ACTH in the right estrogen-producing adrenocortical adenoma as compared to the nonneoplastic adrenal cortex. This case study revealed unique profiles of steroid production in estrogen-producing adrenocortical adenoma associated with concomitant primary aldosteronism. Sequential steroid profiling analysis using LC-MS/MS in combination with AVS can contribute to the diagnosis of various adrenal disorders.