A Rare Case of Cushing's Syndrome Due to Bilateral Cortisol-Secreting Adenomas

Presentation Number: SUN-0783
Date of Presentation: June 22nd, 2014

Atsushi Yasuda*1, Toshiro Seki1, Kazuko Ito2, Masami Seki3, Atsushi Takagi2 and Masafumi Fukagawa1
1Tokai Univ Sch Med, Kanagawa, Japan, 2Tokai University School of Medicine, Kanagawa, Japan, 3Seirei Numazu Hospital, Shizuoka, Japan


Cushing’s syndrome due to bilateral cortisol-secreting adenomas rarely occurs. A 63-year-old man with Cushing’s syndrome due to bilateral adrenocortical adenomas is reported. The patient suffered from diabetes mellitus and hypertension for a decade. He presented with a typical Cushingoid appearance. The serum cortisol level was elevated with loss of the diurnal rhythm and the plasma adrenocorticotropic hormone (ACTH) level was undetectable. Plasma cortisol levels were not suppressed after either low or high dose overnight dexamethasone suppression tests. Computed tomographic scan showed masses in each adrenal gland. Adrenal scintigraphy revealed tracer accumulation in both adrenal glands. We based our preoperative diagnosis of bilateral adrenocortical tumors causing Cushing’s syndrome on the results of endocrinological investigations and imaging findings. Laparoscopic total adrenalectomy has become a standard technique for small adrenal tumors; however, bilateral adrenalectomy results in postoperative adrenal insufficiency, necessitating lifelong steroid replacement. To preserve adrenal function, the left adrenal gland was totally resected, while the right one was partially resected by laparoscopic approach. Hydrocortisone supplementation was initiated at a dose of 30 mg/day. However, symptoms of adrenal insufficiency developed, and adrenal steroid secretion did not respond to exogenous adrenocorticotropic hormone postoperatively. At the 1-year follow-up he was maintained on low-dose hydrocortisone, and with no recurrence of hypercorticolism. The present study comprised a relatively small number of patients and the surgical procedures, whether total/partial adrenalectomy or bilateral total adrenalectomy, applied were not uniform. Total adrenalectomy can cure Cushing’s syndrome, but results in permanent hormone replacement therapy. Bilateral cortisol-secreting tumors are a rare cause of Cushing’s syndrome, and a few cases of bilateral adrenal-preserving surgery were reported. However, our patient developed adrenal insufficiency, after discontinuing his oral cortisone supplement. This report shows that partial adrenalectomy does not necessarily preserve normal adrenocortical function.


Nothing to Disclose: AY, TS, KI, MS, AT, MF