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

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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.