Management of primary bilateral macronodular adrenal hyperplasia causing primary hyperaldosteronism and Cushing's syndrome: Case report and review of the literature

Abstract

Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a very heterogeneous entity. The incidental identification of an increasing number of cases has shifted its clinical expression. Both in terms of cortisol excess and adrenal hyperplasia with mild forms of asymptomatic and oligosymptomatic cases with less impressive imaging phenotypes. Activation of the cAMP/PKA pathway, either due to alteration of various downstream signaling pathways or by aberrantly expressed G protein-coupled receptors, is linked to both cortisol secretion and adrenal growth. Germline mutations in ARMC5 are a frequent genetic defect. We report the case of a 35 -year-old female patient with primary hyperaldosteronism associated with Cushing's syndrome in the setting of macronodular adrenal hyperplasia treated by left adrenalectomy in the first instance and whose evolution was marked by a clinical and biological improvement of hypercorticism and hyperaldosteronism questioning the indication of controlteral adrenalectomy. The appropriate management of PBMAH remains controversial. Bilateral adrenalectomy results in lifelong steroid dependence. And is best reserved for patients with severe Cushing’s syndrome. Unilateral adrenalectomy may be considered in selected patients. In cases where regulation of cortisol secretion is mediated by aberrant receptors, there is some potential for medical therapy