Role of unilateral adrenalectomy in bilateral adrenal hyperplasias with Cushing's syndrome

Primary bilateral adrenocortical hyperplasias are rare forms of pituitary ACTH-independent Cushing's syndrome (CS). They are divided between primary bilateral macronodular adrenal hyperplasia (PBMAH) and micronodular adrenal hyperplasia (MiBAH), which is subdivided in primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease (i-MAD). One of the most debated aspects surrounding these entities is their most appropriate therapy. Although bilateral adrenalectomy (BA) has previously been the most utilized therapy for patients with overt CS, recent studies have indicated that unilateral adrenalectomy (UA) can beeffective inpatientswithPBMAHandsomewithMiBAHwith fewer long-termside effects. Medical therapies can also be used for bridging to surgery or rarely in the long-term for these patients. We reviewthe various degrees of CS resulting from PBMAH and MiBAH, with a special focus on their respective therapies including UA, taking into account the recent pathophysiological and genetics findings. (c) 2021 Elsevier Ltd. All rights reserved.

Automated summary

Primary bilateral adrenocortical hyperplasias are rare forms of pituitary ACTH-independent Cushing's syndrome, with treatment options including bilateral adrenalectomy and unilateral adrenalectomy, as well as medical therapies.