Laparoscopic adrenalectomy for nonfamilial adrenal medullary hyperplasia

Abstract

Background and objectives: Sporadic adrenomedullary hyperplasia (AMH) is characterized by a medical history of hypertension, excessive catecholamine excretion, and histomorphometric evidence of increased adrenomedullary tissue relative to the cortex in the absence of multiple endocrine neoplasia. The aim of this study was to perform a retrospective analysis of patients after laparoscopic adrenalectomy for AMH, an early form of sporadic adrenal medulla-related endocrine hypertension, as well as to update our understanding of the clinical features and management of this clinicomorphologic entity.

Methods: We performed a retrospective review of the medical records of patients operated on between 2007 and 2011 at Reina Sofia University General Hospital, Murcia, Spain, with a diagnosis of AMH. Patient characteristics, diagnostic studies, surgical procedures, and histologic findings were analyzed.

Results: Seven hypertensive patients with intermittent adrenergic crises were found to have AMH (3 men and 4 women; mean age, 44 years). Catecholamine levels were increased. Radiologic studies included 1 or more of the following: magnetic resonance imaging, computed tomography, positron emission tomography imaging with fluorodeoxyglucose, dihydroxyphenylalanine-positron emission tomography-computed tomography, Octreoscan (Mallinckrodt Pharmaceuticals, St. Louis, MO, USA) and (123)I-metaiodobenzylguanidine scintigraphy. Laparoscopic adrenalectomy was performed in all cases. One patient underwent bilateral adrenalectomy because of persistent symptomatology after unilateral adrenalectomy. Surgery was associated with normalization of catecholamine hypersecretion and complete disappearance of symptoms, as well as the reduction or abstention of antihypertensive therapy.

Conclusions: Sporadic AMH is a clinicomorphologic entity that may mimic pheochromocytoma clinically. Recent advances in diagnostic and surgical methods have changed the management and outcome of this unusual disease. Laparoscopic adrenalectomy may be recommended as the gold standard in the treatment of this entity. Definitive diagnosis is provided by histologic study.

Figure 1.

Appearance of the dissection of the left adrenal gland during two different moments of the surgery.