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。最近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。最近提交了两项合作研究的结果，现已发表。与Sunita Agarwal博士，MDB，NIDDK合作，检查了患有胸腺类癌肿瘤的MEN 1患者的分子肿瘤变化，与以前的研究相反，使用我们详细的方法，发现MEN 1基因中存在洛缺失，这也是一个矛盾的领域。与Steve Wank博士和Sumona Bhattacharya，DDB，NIDDK合作的第二项研究表明，可以在服用质子泵抑制剂时进行分泌素测试来诊断ZES，这也是一个有争议的话题。我们的前瞻性研究的见解被用于最近的两个合作共识文件：一个处理的一般诊断，治疗和随访的神经内分泌肿瘤患者的日本神经内分泌学会的成员和欧洲神经内分泌肿瘤网络的成员写的第二个临床实践指南的阐述。这第二篇论文是一份立场文件，提出了NET患者内镜检查的标准化报告方法，ENGLISH将其确定为这些患者治疗中未满足的需求。最后，使用我们的数据库以及文献中的数据，详细分析了用于确定NET/胃泌素瘤患者中可能存在的治疗耐药疾病的预测因素，并发表了其识别中涉及的争议。

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 recently completed . Recently the results of two collaborative studies were submitted and are now published. In collaboration with Dr Sunita Agarwal, MDB , NIDDK the molecular tumoral changes in MEN1 patients with thymic carcinoid tumors was examined and, in contrast to previous studies, using our detailed methods they were found to have LOH in the MEN1 gene, which has also been an area of conflict. A second 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 also has been a controversial subject. Insights from our prospective studies were used in two recent collaborative consensus papers: one dealing with an elucidation of clinical practice guidelines for the general diagnosis, treatment and follow-up of patients with neuroendocrine tumor written with members of the Japanese Neuroendocrine Society and the second with members of the European Neuroendocrine Tumor Network. This second paper was a position paper proposing a standardized reporting approach for endoscopy in patients s with NETs, which was identified by ENETs as an unmet need in the treatment of these patients. Lastly, using data from our database as well as the literature, a detailed analysis of predictive factors for identify the possible presence of treatment resistant disease in patients with NETs/gastrinomas and the controversies involved in their identification was published.