A 25-year-old female, referred because of left hemianopsy, weight gain, fatigue, muscle weakness, and secondary amenorrhea. Physical examination revealed overt Cushingoid features and left temporal hemianopsy. Previously the diagnosis of pituitary macroadenoma (MRI) with suprasellar, infrasellar, and right parasellar propagation was made in regional hospital. Laboratory results showed moderate hypercalcemia with hypophosphatemia and hypercalciuria with inappropriately normal PTH. Unsupressible cortisol during low- and high-dose Dexamethason (Dex) test confirmed the diagnosis of Cushing syndrome (CS), with supressed basal ACTH level pointing to ACTH-independent CS. During high dose Dex test severe headache and visual loss developed due to pituitary apoplexy and urgent trancranial surgery was performed. Chromophobe pituitary adenoma was confirmed by negative immunohistochemistry (LH, FSH, PRL, ACTH, TSH). Clinical and biochemical signs of hypercortisolism persisted after pituitary surgery. Further assesment revealed 4 cm adrenal mass (CT). After the left adrenalectomy, CS resolved and pathohistology confirmed the diagnosis of adrenal cortical adenoma. Substitution therapy with hydrocortisone was instituted. Although MIBI scintigraphy was negative, subtotal parathyroidectomy with total thyroidectomy was performed and primary hyperparathyroidism due to parathyroid gland hyperplasia was diagnosed. Serum calcium and PTH levels normalized. Her periods become regular. The persistence of hypocortisolism with reduced IGF1 concentration one year after surgery, can be explained by secondary hypocortisolism, rather than by prolonged supression of HPA axis. Despite negative family history, genetic screening for MEN 1 mutation is in the course. We presented here a rare case of Cushings in MEN I syndrome due to adrenocortical adenoma.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology