Endocrine Abstracts

Introduction: Bilateral adrenal tumors in patients with adrenal Cushing’s syndrome (CS) are rare, lending to differential diagnosis which includes bilateral macronodular adrenal disease (BMAD), synchronous adrenal adenomas and even adrenocortical carcinoma. The treatment consists of unilateral adrenalectomy, followed by contralateral adrenalectomy, if warranted by persistent significant CS.

Aim: To present a case highlighting unilateral adrenalectomy as an effective treatment of CS in patients with bilateral adrenal tumors.

Case Report: A 54 year old woman with a history of arterial hypertension, and incidentally discovered bilateral adrenal tumors presented to our clinic with minimal clinical signs of CS. Serum cortisol did not suppress after 1 mg dexamethasone overnight, showing mild autonomous cortisol secretion (cortisolemia 3.8 mg/dl). 24-hour urinary free cortisol showed two increased values, midnight serum cortisolemia was mildly elevated and ACTH was suppressed (< 5 pg/ml). Co-secretion of adrenal androgens was absent and 17 HO-progesteron was low. Aldosteronoma and pheochromocytoma were excluded. Family history was negative for endocrine tumors and biochemical screening for hyperparathyroidism and pituitary disease was negative, making MEN1 syndrome unlikely. Adrenal CT scan demonstrated a large heterogenous right adrenal tumor (6/6.75 cm), with native density >10 HU and peripheral calcification and a left adrenal homogeneous macronodule (1.91/2.79 cm) with density >10 HU. We tested cortisol response to stimuli, to detect the expression of aberrant adrenal receptors and observed responses to ortostatism and partially to GnRH analog. Right laparoscopic adrenalectomy was performed and while initial pathology reported an adrenal endothelial vascular cyst, the immunohistochemical diagnosis was of adrenal cortical adenoma with cystic-hemorrhagic changes. Basal cortisolemia was normal and ACTH unsuppressed one week postoperatively and we discontinued glucocorticoid replacement. Later testing demonstrated complete cortisol suppression after overnight 1 mg dexamethasone and normal midnight cortisolemia, consistent with CS remission.

Conclusion: Unilateral adrenalectomy was curative for our patient. Interestingly, adrenal function resumed rapidly. Regular follow-up of the left adrenal nodule is mandated.