BILATERAL ADRENAL MYELOLIPOMAS PRESENTING AS ACUTE ADRENAL INSUFFICIENCY IN AN ADULT WITH CONGENITAL ADRENAL HYPERPLASIA

Abstract

Introduction Adrenal myelolipomas are relatively rare, benign tumors composed of adipocytes and myeloid cells that arise in response to chronic adrenocorticotropic hormone (ACTH) stimulation. We present the case of bilateral adrenal myelolipomas in a patient with untreated congenital adrenal hyperplasia (CAH) presenting with acute adrenal insufficiency and severe virilization. Case A 39-year-old Hispanic adult, who raised as a male, presented with nausea, vomiting, and hypotension. Phenotypically, he was a man of short stature. He was noted to have hyperpigmentation of the skin, gingiva and nail beds. Genital examination revealed micropenis and no palpable testes. Laboratory testing was significant for hyponatremia, hyperkalemia, a low cortisol and elevated ACTH, consistent with primary adrenal insufficiency. An abdominal computerized tomography showed 6×4.5×5 cm right and 2.3×1.4×2.5 cm left adrenal masses with radiological characteristic of myelolipomas. A magnetic resonance imaging of pelvis revealed a...