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Vol. 80, Suppl. 1, 2004
Issue release date: October 2004
Neuroendocrinology 2004;80(suppl 1):23–27

Gastrinomas: Advances in Diagnosis and Management

Jensen R.T.
Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Md., USA

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Gastrinomas causing Zollinger-Ellison syndrome (ZES) are the most common functional, malignant pancreatic endocrine tumors. In this paper, the diagnosis and treatment of these tumors are reviewed, incorporating recent advances in each area. Furthermore, recent advances in their pathology, molecular pathogenesis, and aspects of their localization using somatostatin receptor scintigraphy, as well as their treatment are discussed. Recent data from our NIH prospective studies on patients with ZES are included to illustrate many of these points.

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  1. Roy P, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, Gibril F, Jensen RT: Zollinger-Ellison syndrome: Clinical presentation in 261 patients. Medicine (Baltimore) 2000;79:379–411.
  2. Jensen RT: Zollinger-Ellison syndrome; in Doherty GM, Skogseid B (eds): Surgical Endocrinology: Clinical Syndromes. Philadelphia, Lippincott Williams & Wilkins, 2001, pp 291–344.
  3. Corleto VD, Delle Fave G, Jensen RT: Molecular insights into gastrointestinal neuroendocrine tumors: Importance and recent advances. Dig Liver Dis 2000;34:668–680.
  4. Weber HC, Venzon DJ, Lin JT, Fishbein VA, Orbuch M, Strader DB, Gibril F, Metz DC, Fraker DL, Norton JA, Jensen RT: Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: A prospective long-term study. Gastroenterology 1995;108:1637–1649.
  5. Yu F, Venzon DJ, Serrano J, Goebel SU, Doppman JL, Gibril F, Jensen RT: Prospective study of the clinical course, prognostic factors and survival in patients with longstanding Zollinger-Ellison syndrome. J Clin Oncol 1999;17:615–630.
  6. Corleto VD, Annibale B, Gibril F, Angeletti S, Serrano J, Venzon DJ, Delle Fave G, Jensen RT: Does the widespread use of proton pump inhibitors mask, complicate and/or delay the diagnosis of Zollinger-Ellison syndrome. Aliment Pharmacol Ther 2001;15:1555–1561.
  7. Roy P, Venzon DJ, Feigenbaum KM, Koviack PD, Bashir S, Ojeaburu JV, Gibril F, Jensen RT: Gastric secretion in Zollinger-Ellison syndrome: Correlation with clinical expression, tumor extent and role in diagnosis – A prospective NIH study of 235 patients and review of the literature in 984 cases. Medicine (Baltimore) 2001;80:189–222.
  8. Frucht H, Howard JM, Slaff JI, Wank SA, McCarthy DM, Maton PN, Vinayek R, Gardner JD, Jensen RT: Secretin and calcium provocative tests in the Zollinger-Ellison syndrome: A prospective study. Ann Intern Med 1989;111:713–722.
  9. Norton JA, Jensen RT: Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1). Surg Oncol 2003;12:145–151.
  10. Kwekkeboom D, Krenning EP, deJong M: Peptide receptor imaging and therapy. J Nucl Med 2000;41:1704–1713.
  11. Gibril F, Reynolds JC, Doppman JL, Chen CC, Venzon DJ, Termanini B, Weber HC, Stewart CA, Jensen RT: Somatostatin receptor scintigraphy: Its sensitivity compared with that of other imaging methods in detecting primary and metastatic gastrinomas: A prospective study. Ann Intern Med 1996;125:26–34.
  12. Metz DC, Pisegna JR, Fishbeyn VA, Benya RV, Feigenbaum KM, Koviack PD, Jensen RT: Currently used doses of omeprazole in Zollinger-Ellison syndrome are too high. Gastroenterology 1992;103:1498–1508.
  13. Norton JA, Fraker DL, Alexander HR, Venzon DJ, Doppman JL, Serrano J, Goebel SU, Peghini P, Roy PK, Gibril F, Jensen RT: Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med 1999;341:635–644.
  14. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Jensen RT: Does the use of routine duodenotomy (DUODX) affect rate of cure, development of liver metastases or survival in patients with Zollinger-Ellison syndrome (ZES)? Ann Surg 2004;239:617–626.
  15. Jensen RT: Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1. J Intern Med 1998;243:477–488.
  16. Sutliff VE, Doppman JL, Gibril F, Yu F, Serrano J, Venzon DJ, Jensen RT: Growth of newly diagnosed, untreated metastatic gastrinomas and predictors of growth patterns. J Clin Oncol 1997;15:2420–2431.
  17. Arnold R, Wied M, Behr TH: Somatostatin analogues in the treatment of endocrine tumours of the gastrointestinal tract. Expert Opin Pharmacother 2002;3:643–656.
  18. Shojamanesh H, Gibril F, Louie A, Ojeaburu JV, Bashir S, Abou-Saif A, Jensen RT: Prospective study of the anti-tumor efficacy of long-term octreotide treatment in patients with progressive metastatic gastrinomas. Cancer 2002;94:331–343.

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