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

National Institute Of Diabetes And Digestive And Kidney Diseases

Investigators

Linked publications & trials

Abstract

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. 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 information from our prospective studies was used to help formulate the European Neuroencorme Tumor Networks position papers on unmet needs in patients with pNETs including gastrinomas, as well as other NETs with advanced disease. The ENETs study section for formulating the unmet needs position paper for pNETs was chaired by Dr Jensen and had particular input from the NIH prospective database. Second, a collaborative study with Prof Y-J Chen, Peking University, Beijing China on the potential importance of alterations in chromosome 3p in gastrinomas is now in press, which demonstrated , in contrast to a number of smaller studies, no correlation in this chromosomal area with tumor or clinical prognostic features. Third, now published this year is an in-depth collaborative study with Prof J Norton, Stanford, of the presence of primary gastrinomas in the liver in Zollinger-Ellison syndrome patients followed at NIH and Stanford university . This study showed 3% of patients have a primary in this area. It is an important group because they are the second largest extra-duodenal/pancreatic location of gastrinomas, and their presence has specific management problems, so they need to be identified. Fourth, a two collaborative reviews with Prof J. Norton and colleagues at Stanford University were published this year related to reviewing the latest updates in management of gastrinoma patients as well, as recent advances in the management of advanced disease in these patients as well as all patients with any NET. Lastly, data from our prospective database was used in formulating approaches and recommending changes in the management of pNETs, as well as evaluating, new antitumor treatments with the mTor inhibitor everolimus in NET/pNETs patients and the importance of recent advances in the role of molecular imaging of these tumors. .