Role of Unilateral Aderenalectomy in ACTH‐Independent Macronodular Adrenal Hyperplasia

Abstract

We read with interest an article published recently in the World Journal of Surgery regarding the role of unilateral adrenalectomy in ACTH-independent macronodular adrenal hyperplasia (AIMAH) [1]. In our institution, we have experience with a few cases of AIMAH including the familial type that were all treated by bilateral adrenalectomy [2–4]. Among them, we initially treated one case of AIMAH by unilateral adrenalectomy; however, successive completion adrenalectomy was required owing to the detection of hypersecretion of cortisol in the remaining adrenal gland during follow-up [3]. A 39-year-old Asian woman was admitted to our hospital for evaluation and treatment of AIMAH. She had typical cushingoid features and hypertension. The 24-hr urinary free cortisol was 646 lg (20–90 lg/day). ACTH was not detectable. The computed tomography scan showed 2and 3-cm ovoid masses in both adrenal glands. Stimulation with mixed meal, arginine-vasopressin, luteinizing hormone, b-adrenergic agonist, serotonin, and upright posture did not change her cortisol secretion. Selective adrenal venous sampling was performed according to current recommendations [5]. It revealed a localized hyperfunctioning tumor in the left adrenal gland (Fig. 1). After laparoscopic left adrenalectomy was performed, the hypertension and cushingoid features disappeared, and the 24-hr urinary free cortisol level returned to normal. Thereafter, we have checked serial blood pressure measurements, the 24-hr urinary free cortisol level, and the size of the remaining adrenal gland. Two years after the left adrenalectomy, the patient became hypertensive again with an elevated 24-hr urinary free cortisol level and evidence of enlargement of the right adrenal gland. Completion adrenalectomy was performed laparoscopically. The patient is currently on replacement therapy with physiologic doses of steroid hormones. Many studies are underway to help define the pathophysiology of AIMAH, especially with respect to the underlying mechanisms of adrenal autonomy, including ectopic and eutopic expression of several candidate receptor genes in the adrenal gland [6]. When the molecular pathophysiology of AIMAH is completely understood, medical treatment with specific antagonizing agents or