The Joint Annual Scientific Meetings of the Endocrine Society of Australia and the Society for Reproductive Biology 2018

A case of a large adrenal myelolipoma in a patient with congenital adrenal hyperplasia (#268)

Shekhar Sehgal 1 , Zoe Raos 2 , Michael Croxson 3 , Michael Rodgers 4
  1. Endocrinology , Waitemata DHB , Auckland , NZ
  2. Gastroenterology , Waitemata DHB , Auckland , New Zealand
  3. Endocrinology , Auckland DHB, Auckland City , Auckland , New Zealand
  4. General Surgery , Waitemata DHB , Auckland , New Zealand


Myelolipomas are benign neoplasms of the adrenal glands that consist of both adipose and hematopoietic tissue which are usually small and have a prevalence of 0.1% in autopsy studies (1). Although adrenal masses occur in 11% of all Congenital Adrenal Hyperplasia(CAH) patients, only 6.5% of these tumours are Myelolipomas (2).

Myelolipomas are usually discovered incidentally, and the malignant potential of an incidental adrenal mass is proportional to its size (3). However several case reports of large Myelolipomas are described in patients with CAH (4).


A 42-year-old man with CAH who had reasonable disease control on long term Prednisone therapy presented to his gastroenterologist with severe colitis. Disease control of colitis was not achieved by either steroidal or non steroidal therapy. An incidental 18cm left adrenal mass was found on MR Enterography with  mixed  areas of solid and fat density. A malignant adrenal lesion (liposarcoma) could not be ruled out on imaging characteristics alone. There was no evidence of adrenal hormone hyper secretion on biochemical testing and the mass did not increase in size on short duration serial imaging.  The mass was resected completely together with the spleen and descending colon due to the strong suspicion of adrenal malignancy. Surgical histology revealed an Adrenal  Myelolipoma. He proceeded to have a stormy post operative course


The case highlights that patients with CAH may present with incidental adrenal tumours that are both large and benign such as Myelolipomas. In these patients the size of an adrenal mass lesion alone is a poor predictor of malignant potential. Increased awareness of such cases in CAH patients is required .

  1. 1. Murakami C, Ishibashi M, Kondo M, Oshiro S, Fujita M, Sato S, Kakos M, Fugue H, Mizuguchi K, Yamani T. Adrenal myelolipoma associated with congenital adrenal 21-hydroxylase deficiency. Internal Medicine. 1992;31(6):803-6.
  2. 2. Nermoen I, Rørvik J, Holmedal SH, Hykkerud DL, Fougner KJ, Svartberg J, Husebye ES, Løvås K. High frequency of adrenal myelolipomas and testicular adrenal rest tumours in adult Norwegian patients with classical congenital adrenal hyperplasia because of 21‐hydroxylase deficiency. Clinical endocrinology. 2011 Dec 1;75(6):753-9.
  3. 3. Young Jr WF. The incidentally discovered adrenal mass. New England Journal of Medicine. 2007 Feb 8;356(6):601-10.
  4. 4.Kale G, Pelley EM, Davis DB. Giant myelolipomas and inadvertent bilateral adrenalectomy in classic congenital adrenal hyperplasia. Endocrinology, diabetes & metabolism case reports. 2015;2015.