Natural history of gastro-entero-pancreatic and thoracic neuroendocrine tumors. Data from a large prospective and retrospective Italian epidemiological study: the NET management study.

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