Giant Bilateral Adrenal Myelolipoma with Congenital Adrenal Hyperplasia

Abstract

Myelolipomas are rare and benign neoplasms, predominant of the adrenal glands, consisting of adipose and mature hematopoietic tissue, commonly discovered incidentally with increased use of radiologic imaging. Few cases of giant bilateral adrenal masses are reported, especially in the setting of congenital adrenal hyperplasia (CAH). We report the case of a 39-year-old male with a history of CAH secondary to 21-α hydroxylase deficiency on steroids since childhood, self-discontinued during adolescence, presenting with abdominal distension, fatigue, decreased libido, and easy bruising. Imaging revealed giant bilateral adrenal masses. He subsequently underwent bilateral adrenalectomy found to be myelolipomas measuring 30 × 25 × 20 cm on the left and weighing 4.1 kg and 25 × 20 × 13 cm on the right and weighing 2.7 kg. Adrenal myelolipomas are found to coexist with many other conditions such as Cushing's syndrome, Addison's disease, and CAH. We discuss the association with high adrenocorticotropic hormone (ACTH) states and review the studies involving ACTH as proponent leading to myelolipomas. Massive growth of these tumors, as in our case, can produce compression and hemorrhagic symptoms. We believe it is possible that self-discontinuation of steroids, in the setting of CAH, may have resulted in the growth of his adrenal masses.

Giant Adrenal Myelolipoma Occurring in the Post-Pregnancy Period§

“…Frequently they are unilateral, and smaller than 4 cm, and tend to develop in the adrenal gland (right/left ratio: 3/2). However bilateral and giant adrenal myelolipomas have been also reported in the literature (2,3) . As far as we knew, the largest adrenal myelolipoma cited in the literature was 40 cm in diameter (4) .…”

Case Report: An incidentaloma that catches your eye - adrenal myelolipoma

“…This metaplasia may occur as a response to necrosis, stress, infections, or prolonged adrenocorticotropic hormone (ACTH) stimulation 11, 18 . For example, Al-Bahri et al 19 reported a case of a large bilateral myelolipoma in a 39 year old male with a history of congenital adrenal hyperplasia secondary to a 21-α hydroxylase deficiency treated with steroids starting in childhood. This was later stopped during adolescence with a subsequent myelolipoma development - supporting the theory that ACTH stimulation causes adrenocortical metaplasia.…”

“…It is estimated that 20% of AIs will have subclinical hormone production and these patients represent an at-risk population with greater risk of metabolic disorders and cardiovascular disease 7, 19 . In the present case, the patient’s hormone values were within normal parameters - ruling out subclinical states, including Conn's Syndrome (hyperaldosteronism), Cushing Syndrome or pheochromocytoma.…”

Giant adrenal myelolipoma - clinical spectrum and management: a single centre experience

“…Though rare, bilateral myelolipomas have been described in literature. [7][8][9][10][11][12][13] Myelolipomas are usually of small size often less than 4 cm diameter. Majority of the patients are asymptomatic but it can attain very large size and cause symptoms.…”