Giant Bilateral Myelolipomas in a Patient with Congenital Adrenal Hyperplasia

In 2020, a rare case of giant bilateral adrenal myelolipomas was reported in a 28-year-old female patient with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The patient had a history of poor medical follow-up and non-adherence to her hydrocortisone treatment. She experienced increased hair growth, weight gain, and bilateral flank pain over several years. Imaging revealed large complex cystic masses in her adrenal glands, measuring 19x12x20 cm on the left and 12x11x11 cm on the right. Her initial lab results indicated poorly managed CAH, with elevated levels of ACTH, total testosterone, androstenedione, 17-OH progesterone, DHEA-sulfate, and plasma renin activity. After adjusting her medication and adding dexamethasone, she underwent a bilateral adrenalectomy. The pathology confirmed the masses were myelolipomas, with the left mass measuring 22.3 cm and the right 16.5 cm in diameter. Postoperative lab values showed significant improvement, and the patient reported relief from flank pain. This case underscores the importance of considering myelolipomas in the differential diagnosis of adrenal masses and suggests that surgical removal is appropriate for large, hormonally active, or symptomatic tumors.