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 completed this year. During the year the results of two collaborative studies were submitted and are now in press. One 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 has been a controversial subject. A second study with Dr Sunita Agarwal, MDB , NIDDK reported on the molecular changes in MEN1 patients with thymic carcinoid tumors and showed they have LOH in the MEN1 gene, which has also been an area of conflict. This study is also now in press. During the year information from our prospective studies was used to help write a state-of-the-art review on all aspects of gastrinoma/Zollinger Ellison syndrome for ENDOTEXT, an on line comprehensive review platform widely utilized for treatment/management of patients with endocrine disorders. This database was also used to review current pharmacotherapeutic strategies of the management of patients with functional tumor syndromes. In addition, an European Neuroendocrine Tumor Networks position paper on unmet needs in patients with pNETs including gastrinomas, as well as other NETs with advanced disease was published. The ENETs study section for formulating the unmet needs position paper for pNETs was chaired by Dr Jensen and had input from the NIH perspective database. Lastly, a collaborative surgical study with Prof J. Norton, Dr A.L.Titian and their colleagues at Stanford University is was published dealing with an analysis of results of surgery for locally advanced pNETs.