ECE2014 Oral Communications Adrenal & Thyroid (5 abstracts)
The European Registry on Cushings Syndrome (ERCUSYN) is designed to collect prospective and follow-up data on patients with Cushings syndrome (CS) and currently (Sep 2013) includes 1006 patients (804 F, 202 M; mean age (+S.D.) 44.7±13.3 years) from 57 centers in 28 countries.
Six hundred and sixty one (66%) had pituitary-dependent CS (PIT-CS), 242 (24%) adrenal-dependent CS (ADR-CS), and 103 (10%) CS from other etiologies, including ectopic (ECT-CS). Death occurred in 35 patients (3%), 43% in PIT-CS and 31% in ECT-CS. Urinary free cortisol (UFC) supported the correct diagnosis in 92% PIT-CS, 86% ADR-CS and 100% ECT-CS. Overnight 1 mg dexamethasone suppression test (1 mg DST) reliably identified 87% PIT-CS, 90% ADR-CS, and 96% ECT-CS. Late night salivary cortisol was diagnostic in 92% PIT-CS, 87% ADR-CS, and 93% ECT-CS. Medical treatment was the first therapeutic option in 286 patients, the majority having PIT-CS (74%). Surgery was the first-line treatment in 624 patients, 399 with PIT-CS (64%), 185 with ADR-CS (30%) and 40 with other etiologies (6%). Of 553 PIT-CS patients who underwent transsphenoidal surgery, 376 (68%) experienced short-term remission (within 2 weeks of surgery), whereas 81 (15%) were not cured. Immediate clinical improvement was only observed in 19/95 (20%) patients with normal postoperative cortisol, and did not occur in a subset of 14 of 351 (4%) with postoperative hypocortisolism. Bilateral adrenalectomy was performed in 6%, 38% with ECT-CS. Long-term remission was described in 84% of PIT-CS with available data (mean follow-up, 43+38 months), 87% of ADR-CS (after 34+28 months), and 50% of ECT-CS (after 43+27 months).
After a mean follow-up of 3.5 years >80% of PIT-CS and ADR-CS in ERCUSYN are in long term remission, but only 50% of ECT-CS. ERCUSYN provides an extensive overview of currently used diagnostic and therapeutic procedures in CS at EU level.