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 be effective in patients with PBMAH and some with MiBAH with fewer long-term side effects. Medical therapies can also be used for bridging to surgery or rarely in the long-term for these patients. We review the 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.

原发性双侧肾上腺皮质增生症是垂体不依赖促肾上腺皮质激素的库欣综合征 (CS) 的罕见形式。它们分为原发性双侧大结节性肾上腺皮质增生症 (PBMAH) 和微结节性肾上腺皮质增生症 (MiBAH)，后者又细分为原发性色素性结节性肾上腺皮质病 (PPNAD) 和孤立性微结节性肾上腺皮质病 (i-MAD)。围绕这些实体最具争议的方面之一是它们最合适的疗法。虽然双侧肾上腺切除术 (BA) 以前一直是显性 CS 患者最常用的治疗方法，但最近的研究表明，单侧肾上腺切除术 (UA) 对 PBMAH 和一些 MiBAH 患者有效，并且长期副作用较少。药物疗法也可用于过渡到手术或很少用于这些患者的长期手术。