ECE2015 Eposter Presentations Adrenal cortex (94 abstracts)
Cushings syndrome (CS) during pregnancy is a rare metabolic condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of the overlapping features of fatigue, weight gain, striae, and emotional changes that occur during normal pregnancy. The clinical presentation together with laboratory and imaging findings help to make a diagnosis. However, changes in maternal hormones and their binding proteins complicate assessment of the normal level of glucocorticoid hormones during gestation. CS during pregnancy is attributable most frequently to an adrenal adenoma and to a lesser degree to ACTH hypersecretion from a pituitary adenoma. Furthermore aberrant expression of various hormone receptors in the adrenal glands have been suggested to be involved in the pathogenesis of this condition, in particular the LH receptor. We investigated and treated three pregnant women with ACTH-independent CS and an adrenal tumor. After uncomplicated delivery patient 1 underwent in vivo testing for aberrant hormone receptor expression in the adenoma. Cortisol responses were found after administration of LHRH, human chorionic gonadotropin (hCG), TRH, glucagon, vasopressin, and a standard mixed meal. All patients were treated with laparoscopic adrenalectomy, after which signs and symptoms of hypercortisolism partially resolved. Adrenal tumour tissue of patients 1 and 2 showed positive immunohistochemical staining of LH receptors. Considering the cortisol responses to LHRH and hCG, and the development of CS during pregnancy in these patients, it is likely that ACTH-independent hypercortisolism was induced by the pregnancy-associated rise in hCG levels that activated aberrantly expressed LH receptors in the adrenal adenoma. Remarkably adrenal adenomas may simultaneously express multiple aberrant receptors and individual ligands may play a role in the regulation of cortisol production responsible for pregnancy-induced CS.