The natural history and management of gastrinomas and other malignant pancreatic endocrine tumors (PET's) is largely based on antedotal reports and small series because of their relatively low incidence. Gastrinomas are the most common symptomatic, malignant PET and sufficient numbers of these patients (n=220) have been entered into our protocols to allow systematic assessment. Studies are now underway, evaluating the natural history of these tumors, definition of factors determining prognosis and improved methods to diagnose these tumors. Detailed studies are also underway investigating the management of these tumors including assessment of newer methods to localize tumor extent (endoscopic ultrasound, somatostatin receptor scintigraphy), definition of the role of surgical resection in both limited metastatic disease and patients with no hepatic metastases; the role of aggressive resection in selected patients, and the role of surgery in patients with MEN1. The possible side-effects of longterm acid secretory control are being examined including effects on vitamin B-12 and iron absorption as well as the development of gastric carcinoids. Lastly, medical treatment of advanced disease by chemotherapy, use of interferon, radiation and somatostatin analogues is being examined.

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Intramural Research (Z01)
Project #
1Z01DK053200-07
Application #
6105828
Study Section
Special Emphasis Panel (DDB)
Project Start
Project End
Budget Start
Budget End
Support Year
7
Fiscal Year
1998
Total Cost
Indirect Cost
City
State
Country
United States
Zip Code
Metz, David C; Cadiot, Guillaume; Poitras, Pierre et al. (2017) Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty gastrin assays, sensitive imaging and limited access to acid secretory testing. Int J Endocr Oncol 4:167-185
Ito, Tetsuhide; Igarashi, Hisato; Uehara, Hirotsugu et al. (2013) Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors. Medicine (Baltimore) 92:135-81
Metz, David C; Jensen, Robert T (2008) Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors. Gastroenterology :
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Jensen, Robert T (2006) Pancreatic neuroendocrine tumors: overview of recent advances and diagnosis. J Gastrointest Surg 10:324-6
Berna, Marc J; Hoffmann, K Martin; Long, Scott H et al. (2006) Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National Institutes of Health and comparison with 537 cases from the literature. evaluation of diagnostic criteria, proposal of new Medicine (Baltimore) 85:331-64
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