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。在Zollinger-Ellison综合征（ZES）（胃泌素瘤）患者的长期管理中，争议的主要领域之一是如何成功地管理酸分泌过多。这是因为大多数患者由于MEN 1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。许多小系列和病例报告强调，在医学上控制影响管理的酸性分泌物的难度越来越大。为了解决这个问题，我们分析了303例前瞻性研究的ZES患者长期/终身治疗酸分泌过多的结果。我们的结果显示，随访48年（平均14年）。酸分泌可以在所有情况下得到控制，但只有通过使用已建立的标准评估酸分泌控制并定期重新评估来单独设定药物剂量。确定了预后因素，以确定患者需要剂量变化，这可能是临床上有用的定制药物剂量，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合作共识文件：一份立场文件，提出了NET患者内镜检查的标准化报告方法，这被ENGLISH确定为这些患者治疗中未满足的需求。最后，使用我们数据库中的数据以及文献，与E Wolin教授一起为普通医学教科书Goldman-Cecil Medicine撰写了关于神经内分泌肿瘤的章节。

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.