A case of ACTH-independent macronodular adrenal hyperplasia associated with multiple endocrine neoplasia type 1.

摘要: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized by the occurrence of tumors in parathyroid glands, pancreas, and anterior pituitary. Approximately 30-40% MEN1 patients also have adrenal lesions, such as hyperplasia, benign adenoma, adrenocortical carcinoma. Most cases are hormonally silent. We describe case a 60-year-old man with bilateral macronodular addition to tumors, multiple insulinomas, non-functioning pituitary microadenoma. Endocrinological tests revealed subclinical hypercortisolism; midnight cortisol level rose slightly (8.0 μg/dL), although basal plasma ACTH levels were within normal range (19.5 pg/mL 12.0 μg/dL, respectively). One 8 mg dexamethasone suppression showed 2.3 9.8 respectively. 131I-adosterol scintigraphy under uptake right-sided predominance. The histological features removed right gland consistent ACTH-independent hyperplasia (AIMAH): immunoreactivity 17α-hydroxylase was predominantly observed small compact cells, while that 3β-hydroxysteroid dehydrogenase exclusively expressed large clear cells. glucose-dependent insulinotropic polypeptide (GIP) receptor at high suggesting GIP responsible for development AIMAH. Unilateral lesions detected patient’s 2 children, who presented symptoms. Genetic abnormalities MEN1, p27, p18 genes not found, however, present may provide clue understanding etiology