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Opinion statement

The first goal of therapy is the control of gastric acid hypersecretion using PPIs or high-dose H2R antagonists.

  • The diagnosis of Multiple Endocrine Neoplasia (MEN I) should be established early in the disease.

  • Localization of gastrinoma tumor should be performed using a combination of endoscopic ultrasonography (EUS), somatostatin receptor scintigraphy (SRS), and computerized tomography (CT), or Magnetic Resonance Imaging (MRI).

  • Surgical resection in sporadic ZES should be performed to attempt cure of tumor.

  • Surgery, hormonal, chemotherapy, embolization therapy or therapeutic OctreoScan should be considered in patients with metastatic tumor.

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References and Recommended Reading

  1. Vinayek R: Zollinger-Ellison syndrome. Recent advances in the management of the gastrinoma. Gastroenterol Clin North Am 1990, 19:197.

    PubMed  CAS  Google Scholar 

  2. Berg CL: Zollinger-Ellison syndrome. Med Clin North Am 1991, 75:903.

    PubMed  CAS  Google Scholar 

  3. van Heerden JA: Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type I. Surgery 1986, 100:971.

    PubMed  Google Scholar 

  4. Day JP, Richter JE: Medical and surgical conditions predisposing to gastroesophageal reflux disease. Gastroent Clin N Am 1990, 19(3):587–607.

    CAS  Google Scholar 

  5. Thompson JC: The role of surgery in the Zollinger-Ellison syndrome. Ann Surg 1983, 197:594.

    Article  PubMed  CAS  Google Scholar 

  6. Vinik AI: Controversies in the management of Zollinger-Ellison syndrome. Ann Intern Med 1986, 105:956

    PubMed  CAS  Google Scholar 

  7. Rappaport KD, White JW: 45-year-old man with dermatitis and weight loss. J Mayo Clin Proc 1995, 70(8):785–788.

    CAS  Google Scholar 

  8. Mozell E, Stenzel P, Woltering EA, et al.: Functional endocrine tumors of the pancreas: clinical presentation, diagnosis, and treatment. Curr Probl Surg 1990, 27(6):301–386.

    Article  PubMed  CAS  Google Scholar 

  9. Chandrasekharappa SC, Guru SC, Manickam P, et al.: Positional cloning of the gene for multiple endocrine neoplasia-type 1. Science 1997, 276(5311):404–407.

    Article  PubMed  CAS  Google Scholar 

  10. Guru SC, Goldsmith PK, Burns AL, et al.: Menin, the product of the MEN1 gene, is a nuclear protein. Proc Natl Acad Sci USA 1998, 95(4):1630–1634.

    Article  PubMed  CAS  Google Scholar 

  11. Burgess JR, Greenaway TM, Shepherd JJ: Expression of the MEN-1 gene in a large kindred with multiple endocrine neoplasia type 1. J Intern Med 1998, 243(6):465–470.

    Article  PubMed  CAS  Google Scholar 

  12. Shan L, Nakamura Y, Nakamura M, et al.: Somatic mutations of multiple endocrine neoplasia type 1 gene in the sporadic endocrine tumors. Lab Invest 1998, 78(4):471–475.

    PubMed  CAS  Google Scholar 

  13. Hersey SJ, Sachs G: Gastric Acid Secretion. Physiol Rev 1995, 75:155–218.

    PubMed  CAS  Google Scholar 

  14. Pisegna JR, deWeerth A, Huppi K, Wank SA: Molecular cloning of the human brain and gastric cholecystokinin receptor: structure, functional expression and chromosomal localization. Biochem Biophys Res Comm 1992, 189:296–303.

    Article  PubMed  CAS  Google Scholar 

  15. Yanagisawa K, Yang H, Walsh JH, Tache Y: Role of acetylcholine, histamine and gastrin in the acid response to intracisternal injection of TRH analog, RX 77368, in the rat. Regul Pept 1990, 27:161–170.

    Article  PubMed  CAS  Google Scholar 

  16. Prinz C, Kajimura M, Scott D, et al.: Histamine secretion from rat enterochromaffinlike cells. Gastroenterology 1993, 105:449–461.

    PubMed  CAS  Google Scholar 

  17. Zeng N, Kang T, Wong H, et al.: The pituitary adenylate cyclase activating polypeptide type 1 receptor (PAC1-R) is expressed on gastric ECL cells: evidence by immunocytochemistry and RT-PCR. Ann N Y Acad Sci 1998, 865:147–156.

    Article  PubMed  CAS  Google Scholar 

  18. Sonnenberg A: Testing for Helicobacter pylori in the diagnosis of Zollinger-Ellison syndrome. Am J Gastroenterol 1991, 86:606.

    PubMed  CAS  Google Scholar 

  19. Weber HC, Venzon DJ, Jensen RT, et al.: Studies on the interrelation between Zollinger-Ellison syndrome, Helicobacter pylori, and proton pump inhibitor therapy. Gastroenterology 1997, 112:84–91.

    Article  PubMed  CAS  Google Scholar 

  20. Pisegna JR, Norton JA, Slimak GG, et al.: Effects of curative gastrinoma resection on gastric secretory function and antisecretory drug requirement in the Zollinger-Ellison syndrome. Gastroenterology 1992, 102(3):767–778.

    PubMed  CAS  Google Scholar 

  21. Fass R, Rosen HR, Walsh JH: Zollinger-Ellison syndrome: diagnosis and management. Hosp Pract 1995, 15:73–76, 30(11):79-80.

    Google Scholar 

  22. Stewart CA, Termanini B, Sutliff VE, et al.: Management of the Zollinger-Ellison syndrome in pregnancy. Am J Obstet Gynecol 1997, 176:224–233.

    Article  PubMed  CAS  Google Scholar 

  23. Maton PN: Zollinger-Ellison syndrome. Recognition and management of acid hypersecretion. Drugs 1996, 52(1):33–44.

    PubMed  CAS  Google Scholar 

  24. Frucht H: Use of omeprazole in patients with Zollinger-Ellison syndrome. Dig Dis Sci 1991, 36:394.

    Article  PubMed  CAS  Google Scholar 

  25. Metz DC: Currently used doses of omeprazole in Zollinger-Ellison syndrome are too high. Gastroenterology 1992, 103:1498.

    PubMed  CAS  Google Scholar 

  26. Maton PN: Role of acid suppressants in patients with Zollinger-Ellison syndrome. Aliment Pharmacol Ther 1991, 5(Suppl 1):25.

    PubMed  Google Scholar 

  27. Metz DC, Pisegna JR, Fishbeyn VA, et al.: Currently used doses of omeprazole in Zollinger-Ellison syndrome are too high. Gastroenterology 1992, 103:1498–1508.

    PubMed  CAS  Google Scholar 

  28. Metz DC, Lew E, Forsmark CE, et al.: Gastroenterology 1998, 114(4):G0926.

    Google Scholar 

  29. Pisegna JR, Huang M, Asvar C, et al.: Gastroenterology1998, 114(4):G1065.

    Google Scholar 

  30. Hirschowitz BI: Zollinger-Ellison syndrome: pathogenesis, diagnosis, and management. Am J Gastroenterol 1997, 92(4 Suppl):44S-50S. This review provides an excellent overview on the diagnosis, medical and surgical treatments available. The review covers the use of proton pump inhibitors in the management of gastric acid hypersecretion.

    PubMed  CAS  Google Scholar 

  31. Jensen RT: Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1. J Intern Med 1998, 243(6):477–488. This article provides significant insights in the approach and management of patients with Zollinger-Ellison Syndrome and Multiple Endocrine Neoplasia Type I (MEN I). Issues such as the natural history of these two conditions are discussed. The role of surgical resection is explored. An extensive database of patients at the National Institutes of Health is used to provide the analysis of the best approaches to disease management.

    Article  PubMed  CAS  Google Scholar 

  32. Mignon M, Cadiot G J: Diagnostic and therapeutic criteria in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1. Intern Med 1998, 243(6):489–494. This is an excellent review that provides a straight-forward approach to the management of those subsets of patients presenting with both Zollinger-Ellison Syndrome and Multiple Endocrine Neoplasia Type I (MEN I).

    Article  CAS  Google Scholar 

  33. Norton JA: Gastrinoma: advances in localization and treatment. Surg Oncol Clin N Am 1998, 7(4):845–861. The author is a leading surgical expert in the surgical management of gastrinomas that produce the Zollinger-Ellison Syndrome. The review provides state of the art information on the management of the duodenal and pancreatic tumors involved in this syndrome.

    PubMed  CAS  Google Scholar 

  34. Jensen RT: Should the 1996 citation for Zollinger-Ellison syndrome read: “Acid-reducing surgery in, aggressive resection out”? Am J Gastroenterol 1996, 91(6):1067–1070.

    PubMed  CAS  Google Scholar 

  35. Moertel CG: Phase II trial of doxorubicin therapy for advanced islet cell carcinoma. Cancer Treat Rep 1982, 66:1567.

    PubMed  CAS  Google Scholar 

  36. Moertel CG: Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 1992, 326:519.

    Article  PubMed  CAS  Google Scholar 

  37. Moertel CG: The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med 1994, 120:302.

    PubMed  CAS  Google Scholar 

  38. Thompson W: Malignant islet-cell tumors of the pancreas. World J Surg 1984, 8:940.

    Article  PubMed  CAS  Google Scholar 

  39. Eckhauser FE: Nonfunctioning malignant neuroendocrine tumors of the pancreas. Surgery 1986, 100:978.

    PubMed  CAS  Google Scholar 

  40. Eastman RC: Adriamycin therapy for advanced insulinoma. J Clin Endocrinol Metab 1977, 44:142.

    Article  PubMed  CAS  Google Scholar 

  41. Moertel CG: Phase II trial of doxorubicin therapy for advanced islet cell carcinoma. Cancer Treat Rep 1982, 66:1567.

    PubMed  CAS  Google Scholar 

  42. Mignon M: Current approach to the management of tumoral process in patients with gastrinoma. World J Surg 1986, 10:703.

    Article  PubMed  CAS  Google Scholar 

  43. Howard JM: Gastroenterology 1983, 84:1192.

    Google Scholar 

  44. Pisegna JR, Slimak GG, Metz DC, et al.: An evaluation of human recombinant alpha interferon in patients with metastatic gastrinoma. Gastroenterology 1993, 105:1179–1183.

    PubMed  CAS  Google Scholar 

  45. Ciaccia D, Gress FG: Somatostatin receptor scintigraphy in the Zollinger-Ellison syndrome. Ann Intern Med 1997, 126(9):741–742.

    PubMed  CAS  Google Scholar 

  46. Lebtahi R, Cadiot G, Sarda L, et al.: Clinical impact of somatostatin receptor scintigraphy in the management of patients with neuroendocrine gastroenteropancreatic tumors. Nucl Med 1997, 38(6):853–858.

    CAS  Google Scholar 

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Pisegna, J. Zollinger-Ellison syndrome. Curr Treat Options Gastro 2, 195–203 (1999). https://doi.org/10.1007/s11938-999-0059-5

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  • DOI: https://doi.org/10.1007/s11938-999-0059-5

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