Session: SUN 130-162-Neuroendocrinology
Poster Board SUN-160
Objective: We aimed to evaluate the clinical features of patients with Cushing syndrome with a presumptive diagnosis of ACTH dependent hypercortisolism that had undergone IPSS.
Design: The medical records of 25 patients followed in our instutition between 2003 and 2012 that had undergone IPSS were retrospectively analyzed.
Results: All of the included 25 patients (22 female, 46±14 years) with endogenous hypercortisolism had clinical and laboratory features of ACTH dependent Cushing syndrome, with a more than 50% supression of cortisol in high dose dexamethasone supression test, giving the impression of pituitary Cushing disease. Eighteeen of the patients were being evaluated for typical cushingoid clinical features whereas hypercortisolism was detected in the resting seven patients after evaluation for incidental adrenal mass for subclinical cushing syndrome. Pituitary magnetic resonance imaging (MRI) detected adenoma with variable localisations (n, right:9, left:11, middle:2, no adenoma:2) and diameters (mean:4.8±2.5 mm). During the IPSS procedure, central gradient was found in 20 (80%) patients. In the 17 of the 20 patients that showed central gradient, IPSS was able to lateralize the hypophyseal hypercortisolism focus, all of which undergone transsphenoidal surgery and become clinically remitted. Four of the patients with no central gradient were later on accepted as adrenal cushing syndrome and one patient as ectopic ACTH syndrome due to lung carcinoid behaving as a hypophyseal cushing in diagnostic tests. Remission was achieved in these patients also after excision of the adrenal mass in the first four and the lung mass in the latter. Three patients that were inoperable were started on medical treatment with ketaconazole.
Conclusion: IPSS is a valuable diagnostic aproach for eliciting an accurate diagnosis of Cushing's disease before transphenoidal surgery and is essential to avoid unnecessary surgical interventions. Hypercortisolism of any origin may mimick Cushing disease and IPSS should become the routine diagnostic procedure rather than for selected usage for distinct patient groups.
Disclosure: AC: Consultant, Novartis Pharmaceuticals, Speaker, Novartis Pharmaceuticals, Speaker, Sanofi, Consultant, Sanofi, Speaker, Astra Zeneca, Speaker, Bristol-Myers Squibb, Consultant, Novo Nordisk, Speaker, Novo Nordisk, Speaker, Eli Lilly & Company. Nothing to Disclose: AG, MY, TD, SM, FB
*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm
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