Clinical and endocrinological features of adrenocorticotropic hormone-independent bilateral macronodular adrenocortical hyperplasia

Purpose: We report clinical findings in 5 patients with adrenocorticotropic hormone independent bilateral macronodular adrenocortical hyperplasia. Materials and Methods: In 4 males and 1 female 32 to 61 years old (median a ge 50) we evaluated clinical symptoms, endocrinological and radiological ch aracteristics, treatment modality and postoperative clinical course. Results: All cases presented with some features of Cushing's syndrome. Endo crinological examination revealed autonomous adrenal cortisol production wi th suppressed adrenocorticotropic hormone and a loss in the diurnal circadi an rhythm of plasma cortisol. Abdominal computerized tomography showed bila teral enlargement of the adrenal glands with multiple nodules. (131)Iodine labeled adosterol scintigraphy demonstrated remarkable bilateral uptake by the adrenal glands. The pituitary gland appeared normal on magnetic resonan ce imaging. Open unilateral complete adrenalectomy and contralateral partia l adrenalectomy were performed in patient 1, open bilateral complete adrena lectomy was done in patients 2 and 3, and 2 and 1-stage laparoscopic bilate ral complete adrenalectomy was performed in patients 4 and 5. Single remove d adrenal glands weighed 32 to 108 gm. (median 60). The histological diagno sis was macronodular adrenocortical hyperplasia in all cases. Postoperative followup was 3 to 90 months. Clinical symptoms of Cushing's syndrome disap peared or improved after surgery in all cases. Conclusions: Although adrenocorticotropic hormone independent bilateral mac ronodular adrenocortical hyperplasia. is a rare form of Cushing's syndrome, physicians are advised to consider it when diagnosing and treating cases o f Cushing's syndrome with enlarged bilateral adrenal glands. Bilateral comp lete adrenalectomy is currently recommended as the treatment of choice.