以分阶段肾上腺切除术治疗原发性双侧大结节肾上腺增生一例并文献复习

Staged Adrenalectomy for Primary Bilateral Macronodular Adrenal Hyperplasia: A Case Report and Literature Review

摘要: 原发性双侧大结节肾上腺增生(PBMAH)是内源性库欣综合征的罕见病因，治疗需平衡高皮质醇血症纠正与肾上腺功能保留的关系。本文报告1例PBMAH患者的诊疗过程并结合文献复习。患者为50岁女性，因“发现双侧肾上腺肿物6个月”入院，既往有3年难治性高血压病史，伴体重增加及典型库欣综合征体征。内分泌检测示血浆皮质醇水平显著升高且昼夜节律消失，ACTH水平被抑制；影像学检查提示双侧肾上腺大结节样增生。经多学科会诊后行腹腔镜下右侧肾上腺全切术，术后病理证实为肾上腺皮质结节状增生。首次术后4年患者症状复发，复查示左侧肾上腺结节进展，再次行腹腔镜下左侧肾上腺部分切除术(切除约4/5)，术后病理仍为皮质结节状增生。术后患者激素水平及临床症状得到有效控制。结合文献复习认为，分阶段肾上腺切除术是PBMAH患者的有效个体化治疗策略，可在控制病情的同时最大限度保留肾上腺功能；对于年轻起病、药物控制不佳的高血压患者，需警惕继发性高血压可能，术后终身随访对监测病情复发及代谢并发症至关重要。

Abstract: Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of endogenous Cushing’s syndrome, and its treatment requires balancing the correction of hypercortisolism and the preservation of adrenal function. This article reports the diagnosis and treatment process of a PBMAH patient and reviews relevant literature. A 50-year-old female was admitted to the hospital due to “discovery of bilateral adrenal masses for 6 months”. She had a 3-year history of refractory hypertension, accompanied by weight gain and typical Cushing’s syndrome signs. Endocrine tests showed significantly elevated plasma cortisol levels with loss of circadian rhythm and suppressed ACTH levels; imaging examinations suggested bilateral adrenal macronodular hyperplasia. After multidisciplinary consultation, laparoscopic total right adrenalectomy was performed, and postoperative pathology confirmed adrenal cortical nodular hyperplasia. The patient’s symptoms recurred 4 years after the first operation. Reexamination showed progression of left adrenal nodules, and laparoscopic partial left adrenalectomy (removing about 4/5 of the adrenal gland) was performed again. Postoperative pathology still showed cortical nodular hyperplasia. After the operation, the patient’s hormone levels and clinical symptoms were effectively controlled. Combined with literature review, it is believed that staged adrenalectomy is an effective individualized treatment strategy for PBMAH patients, which can control the disease while maximizing the preservation of adrenal function. For hypertensive patients with early onset and poor drug control, secondary hypertension should be alert. Lifelong follow-up after surgery is crucial for monitoring disease recurrence and metabolic complications.