Abstract
The aim of the present study was to present the treatment observations of patients with adrenocorticotrophic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) from a single medical center. A total of 23 patients with AIMAH were retrospectively reviewed according to the clinical features of the patient. Physical symptoms and biochemical evidence of Cushing’s syndrome (CS) were present in all the cases. High and low dose dexamethasone failed to suppress cortisone secretion on the suppression tests and ACTH levels were low in all the cases. Bilateral enlarged adrenal glands were observed with computed tomography scans in the 23 cases, which were determined to be bilateral adrenal macronodular hyperplasia by pathological analysis. Normal blood pressure was observed in eight patients in the 2–8 years following the bilateral adrenalectomy. A total of 15 cases underwent single side adrenalectomy and their blood pressures markedly decreased three years following surgery; medical treatment was administered to 12 of the 15 patients, while three patients underwent a contralateral adrenalectomy. Nelson’s syndrome was not diagnosed for any of the cases. AIMAH has unique endocrinological, radiological and pathological features, presenting as an independent etiological factor of CS. Diagnosis of AIMAH is usually derived from pathological examination and long term remission may be achieved by unilateral adrenalectomy. Contralateral adrenalectomy should be performed in cases of recurrence, when followed with periodical examination of the symptoms and the serum concentration of cortisol.
Highlights
Adrenocorticotrophic hormone (ACTH)‐independent macronodular adrenal hyperplasia (AIMAH) is a rare disorderCorrespondence to: Dr Chang‐hai Yang, Department of Urology, Tianjin Medical University General Hospital, 154 Anshan Street, Tianjin 300052, P.R
The results demonstrated that plasma cortisol levels were elevated in 20 patients
The levels of urinary free cortisol (UFC) were increased, while the levels of ACTH were decreased in the 23 patients (Table I)
Summary
Patients with AIMAH are diagnosed following incidental radiological observations or the investigation of an adrenal over‐secretion syndrome [4,5]. AIMAH usually presents without symptoms, in a number of cases patients are diagnosed following clinical CS. The most common cause (95% of patients) of AIMAH is adrenocortical adenoma or carcinoma. The majority of the remaining patients have primary pigmented nodular adrenal disease, a syndrome that is characterized by multiple small bilateral pigmented adrenocortical nodules, and is often associated with the Carney complex [6]. A total of AIMAH cases are presented; the patients were admitted to the Tianjin Medical University General Hospital (Tianjin, China) between July 1994 and 2010, and diagnosed by pathology
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