Endocrine Abstracts

Background: Bilateral micronodular adrenal cortical disease (miBACD) is a rare and difficult-to-manage disease. miBACD is one of the causes of Cushing’s syndrome. miBACD is divided into primary pigmented nodular adrenocortical disease (PPNAD) and isolated micronodular adrenocortical disease. Its more common presentation is familial PPNAD, as part of Carney syndrome. We also presented a rare case of miBACD.

Case presentation: A 20-year-old female patient presents to the emergency department with left flank pain and 140/100 mmHg blood pressure. On physical examination, there were purple striae in the abdomen. In the computed tomography scan, an increase in the size of both surreal glands, and a few nodular appearances, in both surreal glands. Cortisol was 28.43 μg/dl, ACTH (adrenocorticotropic hormone) was <10 pg/mI, while other functional screening tests were normal. As a result of suppression tests and 24-hour urine cortisol tests, she was evaluated as having ACTH-independent hypercortisolism. Bone mineral densitometry (BMD) was osteoporotic. She had a left adrenalectomy operation, and the pathology presented as a pigmented nodular adrenocortical disease. Before the operation, the presence of Carney complex and especially cardiac myxomas, was ruled out. In the fifth month after the operation, ketoconazole treatment was started because of the suppression tests. Seven years after the first operation, she complained of severe abdominal pain again. The operation of the other surreal gland was performed and was also operated, and the pathology was the same. She was followed up with hydrocortisone and fludrocortisone replacements.

Conclusions: miBACD is a rare and challenging disease. Management of the disease is critical in multidisciplinary centers. Bilateral adrenalectomy is generally the treatment of choice in patients with overt CS and miBACD, but unilateral adrenalectomy may be considered in cases with asymmetric disease and mild hypercortisolism.