January - March 2013, Volume 9, Issue 1

Sancak S, Altun H., Aydin H., Tukun A. , Mantoglu B., Ender O., Karip B., Okuducu M., Baskent A., Alp T., Memisoglu K

Bilateral Adrenal Myelolipoma in a 46 XX DSD patient with Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency. case report

Acta Endo (Buc) 2013, 9 (1): 109-119
doi: 10.4183/aeb.2013.109

Increased frequency of adrenal tumours and adrenal myelolipoma has been reported in patients with 21-hydroxylase deficiency (21-OHD). Adrenal myelolipoma is an uncommon, benign, biochemically non-functioning tumor and occasionally reported in association with endocrine disorders. Diagnosis of myelolipomas is based on imaging with ultrasonography, CT or MRI being effective in more than 90% of cases. We present a 34-year-old man with massive bilateral adrenal masses which was detected on computed tomography and was diagnosed as 21-hydroxylase deficiency (21-OHD) based on biochemical findings. Computerized tomography of the abdomen demonstrated bilaterally very low-density adrenal masses (16x28 mm on the right side and 91x88 and 33x30 mm on the left side) consistent with adrenal myelolipomas. Since myelolipomas are considered as benign tumors, he was not operated. Tumor size did not increase during two year follow-up periods. It is recommended to the physicians to be aware of increased frequency of benign adrenal tumors that occur frequently in patients with 21-OHD. Untreated CAH with prolonged excessive ACTH stimulation might contribute to the growth of adrenal masses. CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

Keywords: adrenal myelolipoma, Congenital Adrenal Hyperplasia.

Correspondence: Seda Sancak MD, Erenköy, Istanbul, 34070, Turkey, E-mail: drsedasancak@gmail.com