A rare large sized primary bilateral macro-nodular adrenal hyperplasia: a case report

Primary bilateral macro-nodular adrenal hyperplasia (PBMAH) is a rare condition that exhibits various manifestations, from subclinical to severe Cushing syndrome (CS). Bilateral adrenalectomy(BA) is a common treatment especially in patients with overt CS symptoms. This study introduce a patient with uncommon large-sized adrenals about ۱۲ and ۱۰ cm. A ۵۹-year-old diabetic man presented with CS symptoms, including edema, muscle weakness, weight gain, and uncontrolled diabetes mellitus. Laboratory tests confirmed ACTH-independent CS, and imaging revealed macro-nodular lesions in both adrenal glands. The patient underwent BA, resulting in the resolution of primary symptoms without complications. Postoperative follow-up showed significant improvements in blood pressure, blood glucose management, weight loss and other related symptoms. This case demonstrates the successful surgical management of large-sized PBMAH and highlights the absence of a direct association between cortisol level severity and mass size .In spite of reports cited for benefits of unilateral adrenalectomy, BA was our choice to prevent the relapse; since we were faced a symptomatic patient with bilateral large adrenals, it was hard to select the larger adrenal to resect unilaterally. The report contributes to the understanding and guidance for managing similar rare large-sized cases.مقدمه/ بیان مساله : PBMAH exhibits various manifestations, from subclinical to severe CS. BA is a common treatment, but the management of postoperative adrenal insufficiency is a concern. In this report, we introduced a patient who had PBMAH and overt CS with a size of about ۱۲ cm.معرفی بیمار : A ۵۹-year-old male presented with a year-long history of bilateral lower limb edema, which had progressively worsened to involve his face and abdomen over the past ۳ months. He exhibited symptoms of weight gain, decreased libido, easy bruising, depression. The patient had a history of type ۲ diabetes, hypertension, and hyperlipidemia and was taking various medications.Upon examination, blood pressure was high, and he displayed signs of Cushing's stigmata: moon face, buffalo hump, abdominal obesity and severe proximal muscle weakness. Lab tests revealed elevated cortisol level (۲۳.۴۵ μg/dL) , low level of ACTH (<۱ pg/mL) and low dose dexamethasone suppression test (۲۰.۳۱ μg/dL) indicated a diagnosis of ACTH-independent CS. Adrenal ultrasound showed irregular nodules in both adrenal glands, which were further evaluated using thin-section CT imaging; it confirmed bilateral macro-nodular lesions, indicative of PBMAH. Pheochromocytoma and primary aldosteronism were ruled out. BA was performed. The histopathological report confirmed macro-nodular adrenal hyperplasia in the resected glands(left:۱۲*۱۰*۳ cm , right:۱۰*۸*۲.۵ cm). Following the surgery, he experienced no serious complications and was discharged with improved laboratory values and a prescription for corticosteroid replacement therapy. Four months’ follow-up showed significant improvements in blood pressure and glucose management, and ۲۰ kg weight loss was observed.

کلیدواژه ها:

Primary bilateral macronodular adrenal hyperplasia ، PBMAH ، bilateral adrenalectomy ، Cushing's syndrome