Russian Registry of Patients with Tumors of the Hypothalamic-Pituitary Region (OGGO): October 2013 Update for Cushing's Disease
Presentation Number: SAT-0643
Date of Presentation: June 21st, 2014
Liudmila Ya Rozhinskaya*1, Ekaterina A Pigarova2, Zhanna E Belaya1, Larisa K Dzeranova1, Natalia N Molitvoslovova1, Galina A Melnichenko1, Ivan I Dedov1, Oksana Dmitrieva3, Mikhail Chernikov4 and Oggo Concortium5
1Endocrinology Research Centre, Moscow, Russia, 2Endocrinology Research Centre, Moscow, Russian Federation, 3Aston Consulting, Mosсow, Russia, 4Aston Consulting, Mosocw, Russia, 5Federal Districts Head Endocrinologists of Russian Federation, Russia
Introduction: The Russian Registry of patients with tumors of the hypothalamic-pituitary region (OGGO) is a national registry founded in 2004 by Russian Society of Endocrinology and Endocrinology Research Centre as a patient registry for acromegaly, in 2006 it was expanded to collect information on all lesions of the hypothalamic-pituitary region. In 2010 the first fully electronic version and in 2013 a new upgraded electronic online version were implemented. At this point the Registry is actively supported by 22 out of 83 regions of Russia, representing about 1/3 of population.
Materials and Methods: There are overall 5340 patients registered in the OGGO from 2004 to 2013.
Results: The largest part of registered patients constitutes patients with acromegaly (53%), followed by patients with prolactinomas (26%), inactive pituitary tumors (9%), Cushing's disease (6%), mixed secretion pituitary tumors (4%) and other tumors (2%). Prevalence of Cushing's disease in Russian Federation that can be calculated from number of patients registered in the database is >3 patients per 1 000 000 population. Among 431 patients with Cushing's disease 85% are women. Peak incidence is between the ages of 20-30 years. Two percent of patients have got first (highest) degree of disability, 22% - second degree and 12% - third degree, 27% don't have any established degree of disability, and 33% - no data. Seventy percent of patients with Cushing's disease are in remission. Hypopituitarism is present in 34% of patients (hypocortisolism - 70%, hypogonadism - 41%, hypothyroidism - 30%, diabetes insipidus - 20%, GH-deficiency - 10%), visual disturbances due to chiasmatic involvment in 5.3% and neurologic complications in 43% (e.g. headaches – 91%, vertigo – 15%, ptosis – 6%). Surgery was performed in 60% of patients, 31% received radiation therapy and 15.5% - underwent total adrenalectomy. Drug therapy is received by 27% of patients, mainly consisting of ketokonazole > 90%, bromocriptine - 44%, cabergoline - 8%, long acting somatostatin analogs <4%.
Conclusions: Our database indicates that Cushing's disease is a rare and highly disabling disease. The definitive remission rate of 70% is quite promissing as medical treatment choices are still limited.
Disclosure: LYR: Coinvestigator, Novartis Pharmaceuticals, Clinician, Novartis Pharmaceuticals, Coinvestigator, Ipsen, Coinvestigator, Pfizer, Inc.. LKD: Clinical Researcher, Pfizer, Inc.. NNM: Clinical Researcher, Ipsen. Nothing to Disclose: EAP, ZEB, GAM, IID, OD, MC, OC