Endocrine Abstracts

Introduction: We designed a retrospective study to investigate the clinical presentation, laboratory and pathological findings of 14 patients with ACTH-independent macronodular adrenal hyperplasia.

Materials and methods: Diagnose of Cushing’ s syndrome was confirmed by biochemical tests, adrenal hyperplasia was confirmed by pathological examination in all patients.

Results: No supression was observed in overnight, low and high dose dexamethasone supression tests. Thirteen (92.9%) were females. Mean age was 39.71±9.18. ACTH concentrations were 23.20±9.70 (12–40 pg/ml). Two patients (14.3%) were diagnosed incidentally, whereas 12 patients had clinical findings. Two patients had diabetes mellitus (14.3%), eight (57.1%) had hypertension. Patients were found to have dorsocervical fat pad (n:9; 64.3%), central obesity (n:9; 64.3%), striae (n:6;42.9%), plethora (n:7;50%), amenorrhea (n:4;28.6%), acantosis (n:4;28.6%), hirsutismus (n:2;14.3%) and myopathy (n:1;7.1%). One had vertebral fracture during follow-up. One of 14 patients was diagnosed as subclinical cushing syndrome and underwent bilateral adrenalectomy. Seven patients underwent unilateral and seven patients underwent bilateral adrenalectomy. Hypocortisolemia developed in six patients after unilateral adrenalectomy and continued for 12.50±9.29 months. Radiotherapy for hypophysis was performed for four patients (n:1;before unilateral adrenalectomy, n:3;after adrenalectomy). Nelson syndrome developed in two patients against radiotherapy in 9th and 10th years. Eight (57.14%) of 14 patients had macronodular, five (35.71%) had micronodular, and one (7.14%) had primary pigmentated nodular adrenocortical nodules (PPNAD). Compact and clear cells were the most frequent cells in pathologic examination.

Conclusion: We have concluded that patients who underwent adrenalectomy had ACTH-dependent adrenal hyperplasia at first, by long and continuous stimulation of ACTH, bilateral nodular hyperplasia had developed in adrenal glands. Through years, nodules may became autonomous and partially lose ACTH dependence and secreted cortisol continously. Therefore, treatment should be chosen as unilateral or bilateral adrenalectomy.