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

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. 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 . Recently the results of two collaborative studies were submitted and are now published. In collaboration with Dr Sunita Agarwal, MDB , NIDDK the molecular tumoral changes in MEN1 patients with thymic carcinoid tumors was examined and, in contrast to previous studies, using our detailed methods they were found to have LOH in the MEN1 gene, which has also been an area of conflict. A second 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 also has been a controversial subject. Insights from our prospective studies were used in two recent collaborative consensus papers: one dealing with an elucidation of clinical practice guidelines for the general diagnosis, treatment and follow-up of patients with neuroendocrine tumor written with members of the Japanese Neuroendocrine Society and the second with members of the European Neuroendocrine Tumor Network. This second paper was 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 detailed analysis of predictive factors for identify the possible presence of treatment resistant disease in patients with NETs/gastrinomas and the controversies involved in their identification was published.