Natural history of gastro-entero-pancreatic and thoracic neuroendocrine tumors. Data froma large prospective and retrospective Italian Epidemiological study: THE NET MANAGEMENT STUDY
A. Faggiano1,2, P. Ferolla3, F. Grimaldi4, D. Campana5, M. Manzoni6, M.V. Davì7, A. Bianchi8, R. Valcavi9, E. Papini10, D. Giuffrida11, D. Ferone12, G. Fanciulli13, G. Arnaldi14, G.M. Franchi6, G. Francia7, G. Fasola15, L. Crinò3, A. Pontecorvi8, P. Tomassetti5, and A. Colao1
1Department of Molecular and Clinical Endocrinology and Oncology, Section of Endocrinology, University of Naples “Federico II”; 2Endocrinology, National Cancer Institute, Fondazione G. Pascale, Naples; 3Multidisciplinary Group for the Diagnosis and Treatment of Neuroendocrine Tumors, Umbria Regional Cancer Network, Department of Medical Oncology, Department of Internal Medicine and Endocrine Sciences, University of Perugia, Perugia; 4SOC Endocrinology and Metabolism, Azienda Ospedaliero-Universitaria Santa Maria dellaMisericordia di Udine, Udine; 5Department of ClinicalMedicine, Policlinico S. Orsola-Malpighi Hospital, University of Bologna, Bologna; 6Department of Internal Medicine, Neuroendocrine Tumors Unit, San Raffaele Scientific Institute, Milan; 7Clinic of Internal Medicine D, University of Verona, Policlinico GB Rossi, Verona; 8Department of Endocrinology and Metabolic Diseases, Università Cattolica del Sacro Cuore, Rome; 9Endocrinology Division, Arcispedale Santa Maria Nuova, Reggio Emilia; 10Department of Endocrine andMetabolic Diseases, Regina Apostolorum Hospital, Albano - Rome; 11Department of Experimental Oncology,Meditteranean Institute of Oncology, Viagrande, Catania; 12Department of Endocrinology and Medical Sciences and Center of Excellence for Biomedical Research, University of Genova, Genova; 13Dipartimento-Struttura Clinica Medica-Patologia Speciale Medica, Istituto di Clinica Medica, University of Sassari, Sassari; 14Division of Endocrinology, Department of Internal Medicine, Polytechnic University of Marche Region, Ancona; 15Department of Medical Oncology, Azienda Ospedaliero-Universitaria Santa Maria della Misericordia di Udine, Udine, Italy

Background: The few epidemiological data available in literature on neuroendocrine tumors (NET) are mainly based on Registry databases, missing therefore details on their clinical and natural history. Aim: To investigate epidemiology, clinical presentation, and natural history of NET. Design and setting: A large national retrospective survey was conducted in 13 Italian referral centers. Among 1203 NET, 820 originating in the thorax (T-NET), in the gastro-enteropancreatic tract (GEP-NET) or metastatic NET of unknown primary origin (U-NET) were enrolled in the study. Results: 93% had a sporadic and 7% a multiple endocrine neoplasia type 1 (MEN1)-associated tumor; 63% were GEP-NET, 33% T-NET, 4% U-NET. Pancreas and lung were the commonest primary sites. Poorly differentiated carcinomas were <10%, all sporadic. The incidence of NET had a linear increase from 1990 to 2007 in all the centers. The mean age at diagnosis was 60.0±16.4 yr, significantly anticipated in MEN1 patients (47.7±16.5 yr). Association with cigarette smoking and other non-NET cancer were more prevalent than in the general Italian population. The first symptoms of the disease were related to tumor burden in 46%, endocrine syndrome in 23%, while the diagnosis was fortuity in 29%. Insulin (37%) and serotonin (35%) were the most common hormonal hypersecretions. An advanced tumor stage was found in 42%, more frequently in the gut and thymus. No differences in the overall survival was observed between T-NET and GEP-NET and between sporadic and MEN1-associated tumors at 10 yr from diagnosis, while survival probability was dramatically reduced in U-NET. Conclusions: The data obtained from this study furnish relevant information on epidemiology, natural history, and clinico-pathological features of NET, not available from the few published Register studies. (J. Endocrinol. Invest. 35: 817-823, 2012) ©2012, Editrice Kurtis

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