{"title":"1型多发性内分泌瘤中的胃-肠-胰神经内分泌肿瘤:治疗进展","authors":"F. Tonelli, F. Marini, F. Giusti, M. Brandi","doi":"10.2217/IJE-2016-0017","DOIUrl":null,"url":null,"abstract":"Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgical removal of GEP-NETs in MEN1 represents the gold standard curative approach. Conventional medical therapies for sporadic GEP-NETs showed to be effective also in a percentage of MEN1 patients. Innovative medical therapies, that have demonstrated to be effective on sporadic GEP-NETs, still need to be evaluated on MEN1 patients in prospective clinical trials and long-term follow-up. This review resumes current knowledge of MEN1 GEP-NETs, discussing surgical and medical approaches, genetic and molecular bases of tumorigenesis, and presenting novel possible drug therapies.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2000,"publicationDate":"2017-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0017","citationCount":"1","resultStr":"{\"title\":\"Gastro-entero-pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: a therapy update\",\"authors\":\"F. Tonelli, F. Marini, F. Giusti, M. Brandi\",\"doi\":\"10.2217/IJE-2016-0017\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgical removal of GEP-NETs in MEN1 represents the gold standard curative approach. Conventional medical therapies for sporadic GEP-NETs showed to be effective also in a percentage of MEN1 patients. Innovative medical therapies, that have demonstrated to be effective on sporadic GEP-NETs, still need to be evaluated on MEN1 patients in prospective clinical trials and long-term follow-up. This review resumes current knowledge of MEN1 GEP-NETs, discussing surgical and medical approaches, genetic and molecular bases of tumorigenesis, and presenting novel possible drug therapies.\",\"PeriodicalId\":42691,\"journal\":{\"name\":\"International Journal of Endocrine Oncology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.2000,\"publicationDate\":\"2017-03-23\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.2217/IJE-2016-0017\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International Journal of Endocrine Oncology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2217/IJE-2016-0017\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Endocrine Oncology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2217/IJE-2016-0017","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Gastro-entero-pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: a therapy update
Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgical removal of GEP-NETs in MEN1 represents the gold standard curative approach. Conventional medical therapies for sporadic GEP-NETs showed to be effective also in a percentage of MEN1 patients. Innovative medical therapies, that have demonstrated to be effective on sporadic GEP-NETs, still need to be evaluated on MEN1 patients in prospective clinical trials and long-term follow-up. This review resumes current knowledge of MEN1 GEP-NETs, discussing surgical and medical approaches, genetic and molecular bases of tumorigenesis, and presenting novel possible drug therapies.
期刊介绍：
International Journal of Endocrine Oncology is a quarterly, peer-reviewed journal that helps the clinician to keep up to date with best practice in this fast-moving field. The journal highlights significant advances in basic and translational research, and places them in context for future therapy. The journal presents the latest research findings in diagnosis and management of endocrine cancer, together with authoritative reviews, cutting-edge editorials and perspectives that highlight hot topics and controversy in the field. Independent drug evaluations assess newly approved medications and their role in clinical practice. The journal welcomes the unsolicited submission of article proposals and original research manuscripts.
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