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

Unilateral adrenalectomy has been proposed in selected patients with primary bilateral macronodular adrenocortical hyperplasia (PBMAH), but its long-term outcome is unclear. Objective: 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. Design: Retrospective observational study in three German and one Italian academic tertiary care center. Patients and Methods: 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. Results: Baseline clinical and biochemical parameters were comparable in patients with unilat-ADXPBMAH, 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 5 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 CSrelated causes; no deaths occurred in the other two groups (P 5 0.008). Deaths occurred exclusively in patients who were not biochemically controlled after unilateral ADX.