Priorities for improving the management of gastroenteropancreatic neuroendocrine tumors

Priorities for improving the management of gastroenteropancreatic neuroendocrine tumors
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DOI:
10.1093/jnci/djn275
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发表时间:
2008-09-17
影响因子:
10.3
通讯作者:
Snyderwine, Elizabeth
Snyderwine, Elizabeth
中科院分区:
医学1区
文献类型:
--
作者:
Modlin, Irvin M.;Moss, Steven F.;Snyderwine, Elizabeth

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2007年9月举行了国家癌症研究所关于胃肠胰腺神经内分泌和类癌肿瘤的首脑会议,介绍了目前公认的治疗这些肿瘤患者的标准,并确定了需要研究和开发的领域。这些肿瘤在临床和病理上是不同的,通常表现为不明显的症状,导致诊断延误数年,在美国的发病率为2.5/10万到5例/10万。根据疾病的部位和程度,5年存活率在15%到95%之间。本报告概述了会议的主要结论,包括胃胰腺神经内分泌肿瘤的最佳实践诊断和治疗策略,以及确定最需要关注的临床和科学领域。最迫切的需要是公共和医生教育,确定用于早期诊断和治疗监测的分子标记,改进成像方式和分子预测,开发标准化的病理分类系统,以及创建具有肿瘤和实验室数据库的区域专业中心。此外,应建立经过充分验证的神经内分泌肿瘤模型和细胞系,以研究控制其生长和分泌的分子机制,并促进特定治疗方法的开发,这些治疗方法应在精心设计的多中心研究中确定的患者组进行检验。
A National Cancer Institute summit meeting on gastroenteropancreatic neuroendocrine and carcinoid tumors was held in September 2007 to present the currently accepted standards of care for patients with these tumors and to identify areas requiring investigation and development. These tumors are clinically and pathologically heterogeneous, present commonly with obscure symptoms that lead to delays in diagnosis of years, and have an incidence in the United States of 2.5 to 5 cases per 100 000. The 5-year survival rates range between 15% and 95%, depending on the site and extent of disease. This report delineates the main conclusions of the meeting, including the best practice diagnosis and treatment strategies for gastropancreatic neuroendocrine tumors, and the identification of clinical and scientific areas that are most in need of attention. The most pressing needs were public and physician education, identification of molecular markers for early diagnosis and therapeutic monitoring, improved imaging modalities and molecular prognostication, development of a standardized pathological classification system, and creation of regional centers of expertise with tumor and laboratory data banks. In addition, adequately validated neuroendocrine tumor models and cell lines should be established to investigate the molecular mechanisms involved in the control of their growth and secretion, and to facilitate the development of specific therapies that should be examined in well-designed multicenter studies of defined patient groups.