P-017 CUSHING'S SYNDROME DUE TO PRIMARY BILATERAL MACRONODULAR ADRENAL HYPERPLASIA AND METACRONOUS PHEOCHROMOCYTOMA

Abstract Introduction Primary bilateral macronodular adrenal hyperplasia (PBMAH) is an uncommon cause of endogenous Cushing’s syndrome (CS). Pheochromocytoma (PCC) is another adrenal tumor derived from the neural crest and arising in the adrenal medulla. Here, we report a case of recurrent overt CS due to PBMAH, occurring two years after unilateral adrenalectomy, concomitant with a newly developed PCC. Clinical Case A 43-year-old woman was admitted to our clinic with a 30-kg weight gain, proximal muscle weakness, menstrual irregularity, easy bruising, and excessive hair growth on the face and body. She had a history of asthma, essential hypertension, type 2 diabetes mellitus, and hyperlipidemia for approximately 23, 10, 4, and 3 years, respectively. Her medications included inhaled budesonide/formoterol 160/4.5 μg/day, oral metformin 1000 mg/day, perindopril 10 mg/day, lercanidipine 10 mg/day, and atorvastatin 20 mg/day. Laboratory results were compatible with a diagnosis of isolated ACTH-independent CS: imaging revealed bilateral macronodular lesions, and she subsequently underwent right adrenalectomy. Postoperatively, she experienced weight loss and improvement in overall well-being. Two years later, she redeveloped CS accompanied by paroxysmal hypertension. All laboratory findings are summarized in Table 1. The left adrenal gland was removed laparoscopically. Histopathological examination revealed a typical PCC along with macronodular–micronodular hyperplasia in the surrounding adrenal tissue. Conclusion Pheochromocytoma with synchronous ACTH- independent AS originating from the same adrenal gland is very rare. In addition, the progression timeline of the disease and the metachronous emergence of PCC contribute to the distinctiveness of our case. This underscores the importance of a thorough re-evaluation in patients presenting with recurrent ACTH-independent Cushing’s syndrome. Without the preoperative identification of PCC and adequate preparation before the second adrenalectomy, the patient could have experienced a more severe complication, potentially even a fatal outcome.Figure 1:Macroscopy of adrenocortical nodular lesions (yellow arrows) and pheocromocytoma (white arrow). Table 1:Laboratory findingsCS: Cushing's syndrome, DST: dexamethazon supression test, f: fasting, P:plasma, PCC: pheochromocytoma, S: serum, U: urine, * Under treatment with oral prednisolone 10 mg/day.