Therapeutic analysis of unilateral adrenalectomy for Cushing syndrome in patients with adrenocorticotropic hormone independent bilateral macronodular adrenal hyperplasia

Abstract

Objective To investigate the therapeutic outcomes of unilateral adrenalectomy for Cushing syndrome in patients with adrenocorticotropic hormone(ACTH) independent bilateral macronodular adrenal hyperplasia. Methods 22 patients diagnosed with Cushing syndrome caused by ACTH independent bilateral macronodular adrenal hyperplasia from January 2005 to December 2015 were retrospectively concluded. There are 17 male patients and 5 female patients with the median age of 46.5 years. All patients were presented with Cushing syndrome such as moon face, buffalo hump and other typical symptoms of Cushing syndrome. The laboratory tests showed disappearance of cortisol rhythm, elevated 24-hour urinary free cortisol, suppressed or normal ACTH and unsuppressed low dose dexamethasone suppressing test. CT scan showed bilateral macronodular adrenal hyperplasia in all patients. Results All the patients received unilateral adrenalectomy. Pathological results showed adrenal cortex nodular hyperplasia. 16 patients had reexamination in the endocrine department. The median follow-up time was 26(17-118)months for these 16 patients. In the follow-up between 3 to 9 months after surgery, laboratory test showed serum and urinary cortisol level returned to normal range and Cushing syndrome also disappeared in these 16 patients. CT scan showed no recurrence. And no adrenal insufficiency occurred.One patient had recurrence one year after surgery and two patients turned better after surgery but had recurrence at 3 years and 10 years after surgery. Two patients received contralateral adrenalectomy and cortisol hormone replacement therapy after surgery. Another patient with recurrence refused second surgery. No recurrence was observed in other patients. Four patients were followed up by telephone, and all recovered well after surgery. 18 patients had hypertension before surgery and their blood pressure significantly decreased after surgery. Among them 13 cases blood pressure returned to normal range. 5 patients had elevated blood glucose before surgery, and 3 patients′ blood glucose returned to normal range. Conclusions Unilateral adrenalectomy for Cushing syndrome in patients with ACTH independent bilateral macronodular adrenal hyperplasia is safe and effective. Key words: Macronodular adrenal hyperplasia; Cushing syndrome; Unilateral adrenalectomy