PSAT019 A Rare Case of Massive Bilateral Adrenal Myelolipomas in a Patient with Congenital Adrenal Hyperplasia

BACKGROUND: Myelolipomas are rare, benign tumors of the adrenal gland that consist of adipose and hematopoietic tissue. Most present unilaterally and rarely exceed 4 cm, but bilateral and large myelolipomas have been reported. The development of these neoplasms has been associated with congenital adrenal hyperplasia (CAH) (1). CLINICAL CASE: A 44-year-old woman with a history of simple virilizing CAH follows in endocrine clinic. She was diagnosed with CAH at birth and underwent vaginoplasty procedures at age 8 and 16. She has been on glucocorticoid therapy with hydrocortisone or prednisone throughout her life, but has had long gaps in treatment (sometimes up to a year) and reports often skipping doses due to concerns regarding facial swelling. She has not had a menstrual cycle for many years and reports shaving her facial hair daily. Her ACTH has been as elevated as 830 pg/mL, 17-alpha-OH-P has been as elevated as 17740 ng/dL, and testosterone as elevated as 401 ng/dL. She now presents for evaluation of an umbilical hernia. She did not have any abdominal or flank discomfort. CT abdomen and pelvis with contrast was obtained and showed bilateral, massive (approximately 25 cm and 18 cm in the largest dimensions on each side) suprarenal masses thought to most likely represent adrenal myelolipomas. She was referred to endocrine surgery and is undergoing discussions regarding possible adrenalectomy. Since the discovery of these adrenal masses, she has been consistently taking prednisone 5 mg daily. CONCLUSION: This case illustrates the potential role of poorly-controlled CAH in the development of massive adrenal myelolipomas. Many hypothesize chronic ACTH stimulation can cause metaplasia of adrenocortical cells and lead to the development of myelolipomas. Review of the medical literature reveals no clear consensus on the management of these rare neoplasms (2). REFERENCES: (1) Al-Bahri S, Tariq A, Lowentritt B, Nasrallah DV. Giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia. Case Rep Surg. 2014;2014: 728198. doi: 10.1155/2014/728198. Epub 2014 Jul 16. PMID: 25140269; PMCID: PMC4124659. (2) German-Mena E, Zibari GB, Levine SN. Adrenal myelolipomas in patients with congenital adrenal hyperplasia: review of the literature and a case report. Endocr Pract. 2011 May-Jun;17(3): 441-7. doi: 10.4158/EP10340.RA. PMID: 21324823. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.