January - March 2019, Volume 15, Issue 1

Piskinpasa H, Ciftci Dogansen S, Kusku Cabuk F, Guzey D, Sahbaz NA, Akdeniz YS, Mert M

Bilateral Adrenal and Testicular Mass in a Patient with Congenital Adrenal Hyperplasia

Acta Endo (Buc) 2019, 15 (1): 113-117
doi: 10.4183/aeb.2019.113

Introduction. Congenital adrenal hyperplasia (CAH) autosomal recessive disorders characterized by impaired adrenal steroid hormone synthesis. The most common form is 21-hydroxylase deficiency (21OHD). Testicular adrenal rest tumors (TARTs) are benign intratesticular masses that occur in male patients with CAH. TARTs are quite common in patients with 21OHD who were diagnosed late. Case report. A 41-year-old male patient with CAH secondary to 21OHD. The patient was referred to our endocrinology department from the andrology clinic for bilateral adrenal masses. Bilateral orchiectomy had been performed due to bilateral testicular masses and azoospermia two years ago. The pathology was reported as Leydig cell tumor. In hormonal assessment, baseline cortisol levels were low, 17-hydroxyprogesterone levels with baseline and after cosyntropin stimulation test were high. As a result of clinic and laboratory assessment, the patient was diagnosed with simple virilising CAH due to 21OHD and adrenal insufficiency. Then, prednisolone replacement was initiated. Bilateral orchiectomy tissue blocks of the patient were reassessed and were considered TART. Magnetic resonance imaging revealed bilateral adrenal masses with 88x55 mm on the right and 41x22 mm on the left. Laparoscopic right adrenalectomy was applied and pathology was reported as myelolipoma. Follow-up of the mass on the left adrenal gland is ongoing. The patient is monitored under prednisolone and testosterone replacement therapy. Early diagnosis of CAH is very important because of the complications it causes. It should be considered especially for bilateral testicular and/ or adrenal masses. Both fertility and adrenal glands can be protected with an early diagnosis and an early glucocorticoid replacement.

Keywords: Congenital adrenal hyperplasia, Testicular adrenal rest tumors, Myelolipoma, Bilateral adrenal masses.

Correspondence: Hamide Piskinpasa MD, Bakirkoy Dr. Sadi Konuk Training and Research Hospital - Department of Internal Medicine, Division of Endocrinology and Metabolism, Istanbul, 34045, Turkey, E-mail: h_karagoz@hotmail.com