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

胃鞘瘤是伴有或不伴有MEN1的患者中最常见的症状性恶性胰腺神经内分泌肿瘤（pNETs），我们的研究方案中纳入了足够数量的此类患者，以便从广泛的数据库中进行系统评估。此外，通过不同的合作者，这些信息经常被合并或与其他pNETs/NET患者的信息进行比较。最近完成了许多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pNETs /神经内分泌肿瘤治疗的主要临床问题。在佐林格-埃里森综合征（Zollinger-Ellison syndrome， ZES）（胃泌素瘤）患者的长期治疗中，争议的主要领域之一是如何成功地控制胃酸分泌过多。这是因为大多数患者由于MEN1或转移性疾病的存在而无法通过手术治愈，因此需要终身治疗。大量的小系列和病例报告都强调了从医学上控制影响管理的酸炒作分泌的难度越来越大。为了解决这个问题，我们分析了前瞻性研究的303例ZES患者长期/终身治疗酸分泌过多的结果。我们的结果显示了长达48年的随访。（平均-14岁）胃酸分泌均可得到控制，但前提是使用既定标准评估胃酸分泌控制，并定期重新评估，从而单独设定药物剂量。预后因素被确定为确定需要剂量变化的患者，这可能在临床上对调整药物剂量有用，但首先需要进行前瞻性研究。我们前瞻性研究的见解被用于最近的一篇协作共识论文：一篇建议NETs患者内窥镜检查标准化报告方法的立场论文，ENETs认为这是这些患者治疗中未满足的需求。最后，利用我们数据库中的数据以及文献，与E Wolin教授为普通医学教科书《Goldman-Cecil medicine》撰写了关于神经内分泌肿瘤的一章。

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. One of the principal areas of controversy in the longtime management of patients with Zollinger-Ellison syndrome (ZES)(gastrinomas)is how to successfully manage the acid hypersecretion. This occurs because most patients cannot be cured by surgery because of the presence of MEN1 or metastatic disease and thus require life-long treatment. Numerous small series and case reports emphasize the increasing difficulty in controlling medically the acid hype secretion which effects management. To address this question, we analyzed our results of the long-term/lifelong treatment of acid hypersecretion in 303 ZES patients who were prospectively studied. Our results showed with follow-up up to 48 yrs. (mean-14 yrs.) acid secretion could be controlled in all, but only if drug doses were individually set by assessing acid secretory control using established criteria with regular reassessments. Prognostic factors were identified for identifying patients requiring dose changes which could be clinically useful for tailoring drug doses, but first need to be prospectively studied. Insights from our prospective studies were used in a recent collaborative consensus paper: 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 chapter on Neuroendocrine tumors was written with Prof E Wolin for the general medicine textbook, Goldman-Cecil Medicine.