Endocrine Abstracts

Introduction: Cushing’s disease is characterized by an endogenous hypercortisolism related to a pituitary adenoma, its association with an empty sella turcica is exceptionally rare.

Case report: A 55 year old diabetic patient with hypertension was admitted for investigation of Cushing’s syndrome. Clinical examination found facial and truncal obesity with facial erythrosis, capillary fragility with multiple ecchymoses, proximal amyotrophy of the limbs. Biologically, urinary free cortisol was 5 times the normal range, midnight cortisol was elevated to 180 ng/ml and Adrenocorticotropic Hormone (ACTH) was 91 pg/ml confirming the ACTH-dependent nature of Cushing’s syndrome, the high-dose dexamethasone suppression test was positive. Magnetic resonance imaging revealed an intra sellar arachnoidocele with no detectable adenoma. The diagnosis of cushing’s disease was retained and the patient was put on ketoconazole 400 mg/d.

Discussion: Imaging-negative cushing’s disease is not uncommon, the association with an empty sella turcica is a major challenge and could be explained by the apoplexy of a pituitary adenoma. Catheterization of the petrous sinuses remains the gold standard for topographic diagnosis. Transsphenoidal surgery is the first-line treatment but is not without risks. Medical treatment allows control of hypercortisolism in case of failure of surgery or while waiting for the appearance of an adenoma on imaging.

References: 1. Philippon et al. / Annales d’Endocrinologie 74 (2013) S 23–32.

2. G.U Mehta et al. Cushing disease and empty sella, J Neurosurg/ Vol 121/ Sept 2014.