Adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia-report of seven cases report and review of literature / 解放军医学杂志

ABSTRACT

Objective To study the characteristics of clinical and laboratory findings in patients with adrenocorticotropic hormone(ACTH)independent macronodular adrenocortical hyperplasia(AIMAH).Methods The clinical and laboratory findings of 7 cases of AIMAH admitted between 1997 and 2005 were reviewed retrospectively.Results 5 of the 7 patients showed typical clinical features of Cushing's syndrome,including moon face,central obesity,hypertension,etc.A part of patients had hypokalemia(43%)and secondary diabetes mellitus(71%).There was lowering of plasma ACTH levels,and an increased cortisol secretion or disturbance in or circadian rhythm.In 6 patients,a low dose or high dose of dexamethasone suppression tests failed to suppress cortisol secretion.CT scan showed bilateral macronodular adrenal hyperplasia.Pituitary MR imaging for pituitary was negative in 6 patients.Unilateral total adrenalectomy was performed in 2 cases.4 cases were treated by bilateral adrenalectomy.One patient died of stroke before operation.5 cases received glucocorticoid replacement therapy after operation.Pathologic examination showed diffuse yellow or brown nodules measuring 0.3 cm～7.0 cm in diameter in all the specimems of 6 patients who undement operation.There was no recurrence in all the patients ofter surgery during the follow-up period.No Nelson syndrome occurred in these 6 patients postoperatively.Conclusion AIMAH is a rare cause of Cushing's syndrome with unique clinical,CT image and pathological findings.Bilateral adrenalectomy in one stage or in stages,or unlateral adremectomy is indicated on the basis of clinical and CT findings.