{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:32:44Z","timestamp":1760189564141},"reference-count":36,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,4,20]],"date-time":"2017-04-20T00:00:00Z","timestamp":1492646400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2017,4,20]],"date-time":"2017-04-20T00:00:00Z","timestamp":1492646400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["World j. surg."],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>The multiple endocrine neoplasia type 1 syndrome (MEN1) natural history is poorly evaluated, and few single\u2010institution experiences about hereditary gastroenteropancreatic neuroendocrine tumors (GEP\u2010NET) are reported. Our purpose is to analyze the role of GEP\u2010NET in MEN1\u2010related death, as well as the behavior of these lesions during follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The study population consists of 77 patients diagnosed with MEN1 GEP\u2010NET, regularly followed up since 1990. Extensive clinical data were prospectively recorded. Statistical analysis was performed both on the whole population of 77 patients and on two subgroups including patients who, during the long lasting study period, underwent GEP\u2010NET surgery (50\u00a0pts) and who did not (27\u00a0pts), respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty\u2010five males (32.5%) and 52 females (67.5%) were enrolled. Sixty\u2010four patients had MEN1 family history (83.1%), and genetic mutation was detected in 67 cases (87%). The mean age at GEP\u2010NET diagnosis was 41.4 years (SD = 13.6); 16 patients (20.8%) had GEP\u2010NET diagnosed before age 30 and 12 cases (15.6%) before 1996. The mean interval time between MEN1 diagnosis and GEP\u2010NET detection was 5.7 years (range \u221211\/37; SD = 8.1 years). Overall, the mean follow\u2010up time from MEN1 diagnosis was 15.8 years (SD = 9.7 years) and from GEP\u2010NET diagnosis was 9.6 years (SD = 6.9 years). Gastrinoma was the most frequent functioning GEP\u2010NET and pancreatoduodenectomy the most adopted surgery. GEP\u2010NET progression affected 12 patients within the non\u2010surgical group, while 18 subjects developed progression after surgery.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Our single\u2010center data provide information on epidemiologic, clinical and pathological features of GEP\u2010NET in MEN1\u00a0making possible to clarify their natural history.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00268-017-4019-2","type":"journal-article","created":{"date-parts":[[2017,4,20]],"date-time":"2017-04-20T16:52:35Z","timestamp":1492707155000},"page":"2312-2323","update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Natural History of MEN1 GEP\u2010NET: Single\u2010Center Experience After a Long Follow\u2010Up"],"prefix":"10.1002","volume":"41","author":[{"given":"Francesco","family":"Giudici","sequence":"first","affiliation":[{"name":"Department of Surgery and Translational Medicine University of Florence Largo Brambilla 3 50139 Florence Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiziana","family":"Cavalli","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine University of Florence Largo Brambilla 3 50139 Florence Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Giusti","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine University of Florence Largo Brambilla 3 50139 Florence Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giorgio","family":"Gronchi","sequence":"additional","affiliation":[{"name":"Department of Surgery and Translational Medicine University of Florence Largo Brambilla 3 50139 Florence Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giacomo","family":"Batignani","sequence":"additional","affiliation":[{"name":"Department of Surgery and 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