A case of Adrenocoricotrophic hormone-independent bilateral adrenocortical macronodular hyperplasia concomitant with primary aldosteronism

作者：Tokumoto Mao; Onoda Naoyoshi^*; Tauchi Yukie; Kashiwagi Shinichiro; Noda Satoru; Toi Norikazu; Kurajoh Masahumi; Ohsawa Masahiko; Yamazaki Yuto; Sasano Hironobu; Hirakawa Kosei; Ohira Masaichi

来源：BMC Surgery, 2017, 17(1): 97.

DOI：10.1186/s12893-017-0293-z

• ACTH - Independent bilateral adrenocortical macronodular hyperplasia
• Primary aldosteronism
• Laparoscopic unilateral adrenolectomy
• Preclinical Cushing's syndrome

Background: Adrenocoricotrophic hormone (ACTH) -independent bilateral adrenocortical macronodular hyperplasia (AIMAH) is a rare cause of Cushing's syndrome, and is characterized by bilateral adrenal hyperplasia. However, Primary aldosteronism (PA) is a relatively common adrenal disease.
Case presentation: A 56-year-old man who has been treated hypertension and diabetes mellitus was detected low plasma potassium level with an elevated level of plasma aldosterone concentration and bilateral adrenal swelling. Endocrinological examinations showed autonomous secretion of cortisol and aldosterone, with suppression of plasma ACTH level and renin activity. A selective adrenal venous sampling demonstrated that left adrenal gland was responsible for aldosterone hypersecretion. He was diagnosed preclinical Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH) associated with aldosterone producing adenoma of the left adrenal gland. A laparoscopic left adrenalectomy was performed.
Conclusion: The resected adrenal specimen histologically consisted with a diagnosis of AIMAH. Moreover, tiny cell clusters positive immunostaining for aldosterone synthase was revealed. This is a rare case of AIMAH accompanied by preclinical Cushing's syndrome and primary aldosteronism.

• 出版日期2017-9-6