Coexistence of Bilateral Giant Adrenal Myelolipomas and Congenital Adrenal Hyperplasia: A Case Report

Adrenal myelolipomas (AMs) are rare and benign neoplasms, consisting of adipose and mature hematopoietic tissue. They are commonly discovered incidentally with increased use of radiologic imaging. A small number of giant bilateral adrenal masses are reported, particularly in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 36-year-old male with a history of CAH on steroids since childhood, self-discontinued shortly after diagnosis, presenting mainly with abdominal distension and pain besides infertility. Imaging revealed giant bilateral adrenal masses. Subsequently, he underwent bilateral adrenalectomy, and the surgical pathology report revealed myelolipomas measuring 39×17×8 cm on the left and weighing 4050 grams and 28×16×5 cm on the right and weighing 1702 grams. AMs are found to coexist with many other conditions such as Cushing’s syndrome, pheochromocytoma, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as a stimulator leading to myelolipomas. This case report highlights the proper history taking and biochemical evaluation for early detection and intervention to avoid catastrophic consequences.