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

胃腺瘤是患有或不患有MEN1的患者中最常见的有症状的恶性胰腺神经内分泌肿瘤(PNETs)，我们已将足够数量的此类患者纳入我们的方案，以便从广泛的数据库进行系统评估。此外，对于不同的合并症患者，这一信息经常被合并或与其他pNET/Net患者的信息进行比较。今年完成了多项不同的研究以及各种合作指南/论文/研究，这些研究涉及分析胃泌素瘤和其他PNETs/神经内分泌肿瘤的主要临床问题。在这一年里，我们的前瞻性研究信息被用来帮助制定两份欧洲神经内分泌肿瘤网络立场文件，内容是包括胃泌素瘤在内的PNETs患者以及其他患有晚期疾病的NETs患者的未得到满足的需求。制定PNETS未满足需求立场文件的ENETS研究部分由Jensen博士担任主席，并从NIH预期数据库中获得了特别的意见。第二，与北京大学中国教授合作的一项关于胃泌素瘤中染色体3p改变的潜在重要性的研究现已发表，与一些较小的研究相反，该研究表明，该染色体区域与肿瘤或临床预后特征没有相关性。第三，与斯坦福大学的J.Norton教授、A.L.Titian博士和他们的同事合作的一项外科研究已进入提交的最后阶段，该研究涉及对当地先进的PNETs的手术结果进行分析。最后，我们使用前瞻性数据库中的数据与文献中的数据进行比较，以制定和确定在PNETs治疗中影响胰腺神经内分泌肿瘤患者总存活率和手术结果的预后因素。

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 colloborators this information is often combined or compared to that in other pNET/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 information from our prospective studies was used to help formulate two European Neuroendocrine Tumor Networks position papers on unmet needs in patients with pNETs including gastrinomas, as well as other NETs with advanced disease. The ENETs study section for formulating the unmet needs position paper for pNETs was chaired by Dr Jensen and had particular input from the NIH prospective database. Second, a collaborative study with Prof Y-J Chen, Peking University, Beijing China on the potential importance of alterations in chromosome 3p in gastrinomas is now published, which demonstrated , in contrast to a number of smaller studies, no correlation in this chromosomal area with tumor or clinical prognostic features. Third, a collaborative surgical study with Prof J. Norton, Dr A.L.Titian and their colleagues at Stanford University is in final stages of submission dealing with an analysis of results of surgery for locally advanced pNETs. Lastly, data from our prospective database compared with data from the literature was used in formulating and identifying prognostic factors on overall survival and surgical outcomes in patients with pancreatic neuroendocrine tumors in the management of pNETs.