Long-Term Outcome of Primary Bilateral Macronodular Adrenocortical Hyperplasia After Unilateral Adrenalectomy

• DOI: 10.1210/jc.2018-02204
• Publication Date: 2019-03-07T09:06:24Z
• Authors: Andrea Osswald; Marcus Quinkler; Guido Di Dalmazi; Timo Deutschbein; German Rubinstein; Katrin Ritzel; Stephanie Zopp; Jerome Bertherat; Felix Beuschlein; Martin Reincke
• Keywords: Adrenal Cortex Diseases; Adult; Male; 1303 Biochemistry; Hydrocortisone; 10265 Clinic for Endocrinology and Diabetology; 610 Medicine & health; 1308 Clinical Biochemistry; 2704 Biochemistry (medical); 03 medical and health sciences; 0302 clinical medicine; Humans; Cushing Syndrome; Aged; Retrospective Studies; Hyperplasia; PBMAH, primary bilateral macronodular adrenocortical hyperplasia, adrenalectomy, adrenal Cushing’s syndrome, hypercortisolism; Adrenalectomy; Middle Aged; Adrenal Cortex Neoplasms; 1310 Endocrinology; 3. Good health; 2712 Endocrinology, Diabetes and Metabolism; Treatment Outcome; Adrenocortical Adenoma; Adrenal Cortex; Quality of Life; Female

Unilateral adrenalectomy has been proposed in selected patients with primary bilateral macronodular adrenocortical hyperplasia (PBMAH), but its long-term outcome is unclear.The aim of this study was to analyze long-term clinical and biochemical outcomes of unilateral adrenalectomy vs bilateral adrenalectomy in patients with PBMAH in comparison with the outcome of cortisol-producing adenoma (CPA) treated with unilateral adrenalectomy.Retrospective observational study in three German and one Italian academic tertiary care center.Twenty-five patients with PBMAH after unilateral adrenalectomy (unilat-ADX-PBMAH), nine patients with PBMAH and bilateral adrenalectomy (bilat-ADX-PBMAH), and 39 patients with CPA and unilateral adrenalectomy (unilat-ADX-CPA) were included.Baseline clinical and biochemical parameters were comparable in patients with unilat-ADX-PBMAH, bilat-ADX-PBMAH, and unilat-ADX-CPA. Directly after surgery, 84% of the patients with unilat-ADX-PBMAH experienced initial remission of Cushing syndrome (CS). In contrast, at last follow-up (median, 50 months), 32% of the patients with unilat-ADX-PBMAH were biochemically controlled compared with nearly all patients in the other two groups (P = 0.000). Adrenalectomy of the contralateral side had to be performed in 12% of the initial patients with unilat-ADX-PBMAH. Three of 20 patients with unilat-ADX-PBMAH (15%) died during follow-up, presumably of CS-related causes; no deaths occurred in the other two groups (P = 0.008). Deaths occurred exclusively in patients who were not biochemically controlled after unilateral ADX.Our data suggest that unilateral adrenalectomy of patients with PBMAH leads to clinical remission and a lower incidence of adrenal crisis but in less sufficient biochemical control of hypercortisolism, potentially leading to higher mortality.