Diagnosis and treatment of adrenal medullary hyperplasia

Abstract

To explore the clinical characters of adrenal medullary hyperplasia (AMH). The clinical data of 7 cases of AMH admitted from 1996 to 2013 were analyzed with a review of literature. There were 3 males and 4 females with a median age of 36 (23-69) years. All had hypertension at first diagnosis and 2 were paroxysmal. Computed tomography (CT) scan revealed unilateral adrenal nodule in all cases, including left (n = 5) and right (n = 2) side. The diameter of nodules was 0.9 cm to 3.0 cm.Hypokalemia was found in 2 cases. And 24-hour output of urine catecholamine was higher than normal level in 2 cases. An abnormal rise of 24-hour output of urine free cortisol was found in 1 case with a lower level of adrenocorticotropic hormone (ACTH) simultaneously while a lower level of plasma renin activity in vertical position was found in another case. Three patients received (131)I-meta-iodobenzylguanidine scan and one had positive result. Pre-operative diagnosis included pheochromocytoma (n = 2), adrenocortical adenoma (n = 3) (2 aldosterone-producing adenomas, 1 Cushing adenoma) and undetermined adrenal tumor (n = 2). All underwent surgery, including unilateral adrenalectomy (n = 4) and resection of adrenal disease (n = 3). All cases had a pathologic diagnosis of AMH. One had concurrent Cushing adenoma while another with cortical hyperplasia.One case achieved normotension postoperatively while others showed varying decreases of blood pressure, but remained hypertensive. With a common feature of hypertension, AMH is difficult to diagnose preoperatively. CT scan has a certain diagnostic significance and a definite diagnosis depends on pathological examination; because of inaccurate preoperative and intraoperative diagnosis, the extent of surgery may be incomplete and results in unsatisfied outcomes.