Diagnosis, Natural History, Management, tumor biology of Gastrinomas/PETs/Neuroendocrine tumors

胃泌素瘤是最常见的症状，恶性胰腺神经内分泌肿瘤（pNET）的患者有或没有MEN 1和足够数量的这些患者已经进入我们的方案，允许系统评估从一个广泛的数据库。此外，在各种合作者的帮助下，这些信息通常会与其他pNET/NET患者的信息相结合或进行比较。今年完成了一些不同的研究以及各种合作指南/论文/研究，涉及分析胃泌素瘤和其他pNET/神经内分泌肿瘤管理中的主要临床问题。在这一年里，提交了两项合作研究的结果，目前正在付印。与Steve Wank博士和Sumona Bhattacharya，DDB，NIDDK合作的一项研究表明，可以在服用质子泵抑制剂的同时进行分泌素测试以诊断ZES，这一直是一个有争议的话题。Sunita Agarwal博士，MDB，NIDDK的第二项研究报告了患有胸腺类癌肿瘤的MEN 1患者的分子变化，并显示他们在MEN 1基因中存在洛缺失，这也是一个冲突的领域。这份研究报告目前也在印刷中。 在这一年中，我们的前瞻性研究的信息被用于帮助ENDOTEXT撰写关于胃泌素瘤/Zollinger Ellison综合征各个方面的最新综述，ENDOTEXT是一个广泛用于内分泌疾病患者治疗/管理的在线综合综述平台。该数据库还用于审查功能性肿瘤综合征患者管理的当前药物治疗策略。 此外，欧洲神经内分泌肿瘤网络发表了一份关于pNET患者（包括胃泌素瘤）以及其他晚期NET患者未满足需求的立场文件。制定pNET未满足需求的立场文件的ENERGY研究部分由詹森博士主持，并从NIH观点数据库中获得了输入。最后，与斯坦福大学的J. Norton教授、A. L. Titian博士及其同事合作的一项外科研究发表，该研究分析了局部晚期pNET的手术结果。

Gastrinomas are the most common symptomatic, malignant pancreatic neuroendocrine tumor (pNETs) in patients with or without MEN1 and sufficient numbers of these patients have been entered into our protocols to allow systematic assessment from an extensive database. Furthermore, with various collaborators this information is often combined or compared to that in other pNETs/NET patients. A number of different studies as well as various collaborative guidelines/papers/studies involved in the analyses of major clinical problems in the management of gastrinomas and other pNETs /neuroendocrine tumors were completed this year. During the year the results of two collaborative studies were submitted and are now in press. One study in collaboration with Dr Steve Wank and Sumona Bhattacharya, DDB, NIDDK demonstrated that secretin testing for diagnosis of ZES could be performed while taking proton pump inhibitors which has been a controversial subject. A second study with Dr Sunita Agarwal, MDB , NIDDK reported on the molecular changes in MEN1 patients with thymic carcinoid tumors and showed they have LOH in the MEN1 gene, which has also been an area of conflict. This study is also now in press. During the year information from our prospective studies was used to help write a state-of-the-art review on all aspects of gastrinoma/Zollinger Ellison syndrome for ENDOTEXT, an on line comprehensive review platform widely utilized for treatment/management of patients with endocrine disorders. This database was also used to review current pharmacotherapeutic strategies of the management of patients with functional tumor syndromes. In addition, an European Neuroendocrine Tumor Networks position paper on unmet needs in patients with pNETs including gastrinomas, as well as other NETs with advanced disease was published. The ENETs study section for formulating the unmet needs position paper for pNETs was chaired by Dr Jensen and had input from the NIH perspective database. Lastly, a collaborative surgical study with Prof J. Norton, Dr A.L.Titian and their colleagues at Stanford University is was published dealing with an analysis of results of surgery for locally advanced pNETs.