Endocrine Abstracts

Introduction: The empty sella syndrome (ESS) is defined as the penetration of the subarachnoid space into intrasellar region. In ESS pituitary hormonal function is usually normal, but several, mostly subtle, hormonal abnormalities have been also reported. The coexistence of Cushing’s disease and primary empty sella is very rare.

Objective: The presentation of an exceptional case of a patient with Cushing’s disease associated with primary empty sella.

Case report: A 70-year-old woman with typical clinical signs of hypercortisolism observed from 11 years. She presented visceral obesity, diabetes and severe hypertension (diagnosed as metabolic syndrome), plethora, thin skin, hemorrhagic diathesis, proximal limbs muscle atrophy, and weakness. Endocrine evaluation of adrenocortical axis proved hypercortisolism of pituitary origin with a loss of ACTH (28.48–28.2 pg/ml) and cortisol (30.0–18.8 μg/dl) circadian rhythms, increased urinary cortisol excretion (693 μg/dl per day), a lack of cortisol suppression after low dexamethasone dose and the presence of suppression after high dexamethasone dose. MRI disclosed subarachnoid cyst (18×16×15 mm) and an empty sella. The pituitary was compressed with the altitude of its anterior lobe of about 3 mm. Abdominal CT revealed bilateral homogenous adrenal hypertrophy without focal changes. Despite of a lack of visible microadenoma on MRI scans, on the base of clinical and laboratory findings, the patient was prepared to neurosurgery with a steroidogenesis inhibitor – ketoconazole. A pituitary microadenoma was removed by the transsphenoidal approach. A postoperative pathologic exploration revealed densely granulated corticotroph adenoma. After the neurosurgery, the patient experienced severe secondary hypoadrenalism and hydrocortisone replacement treatment was administered. Other pituitary trophic hormones remained normal.

Conclusion: It is worth emphasizing that metabolic syndrome together with an empty sella syndrome did not rule out a pituitary corticotroph adenoma.