Zollinger-Ellison syndrome

References

Key articles

Falconi M, Eriksson B, Kaltsas G, et al. ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103(2):153-71.Full text  Abstract

Rossi RE, Elvevi A, Citterio D, et al. Gastrinoma and Zollinger Ellison syndrome: a roadmap for the management between new and old therapies. World J Gastroenterol. 2021 Sep 21;27(35):5890-907.Full text  Abstract

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.Full text  Abstract

Reference articles

1. Nieto JM, Pisegna JR. The role of proton pump inhibitors in the treatment of Zollinger-Ellison syndrome. Expert Opin Pharmacother. 2006 Feb;7(2):169-75. Abstract

2. Hung PD, Schubert ML, Mihas AA. Zollinger-Ellison syndrome. Curr Treat Options Gastroenterol. 2003 Apr;6(2):163-70. Abstract

3. Norton JA. Neuroendocrine tumors of the pancreas and duodenum. Curr Probl Surg. 1994 Feb;31(2):77-156. Abstract

4. Metz DC, Sostek MB, Ruszniewski P, et al. Effects of esomeprazole on acid output in patients with Zollinger-Ellison syndrome or idiopathic gastric acid hypersecretion. Am J Gastroenterol. 2007 Dec;102(12):2648-54. Abstract

5. Corleto DV, Annibale B, Gibril F, et al. Does the widespread use of proton pump inhibitors mask, complicate and/or delay the diagnosis of Zollinger-Ellison syndrome? Aliment Pharmacol Ther. 2001 Oct;15(10):1555-61.Full text  Abstract

6. Metz DC, Cadiot G, Poitras P, et al. 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. 2017;4(4):167-85.Full text  Abstract

7. Pisegna JR. The effect of Zollinger-Ellison syndrome and neuropeptide-secreting tumors on the stomach. Curr Gastroenterol Rep. 1999 Dec;1(6):511-7. Abstract

8. Alexakis N, Neoptolemos JP. Pancreatic neuroendocrine tumors. Best Pract Res Clin Gastroenterol. 2008;22(1):183-205. Abstract

9. Mortellaro VE, Hochwald SN, McGuigan JE, et al. Long-term results of a selective surgical approach to management of Zollinger-Ellison syndrome in patients with MEN-1. Am Surg. 2009 Aug;75(8):730-3. Abstract

10. Benya RV, Metz DC, Venzon DJ, et al. Zollinger-Ellison syndrome can be the initial endocrine manifestation in patients with multiple endocrine neoplasia-type I. Am J Med. 1994 Nov;97(5):436-44. Abstract

11. Jensen RT. Gastrinomas: advances in diagnosis management. Neuroendocrinology. 2004;80(suppl 1):23-7. Abstract

12. Falconi M, Eriksson B, Kaltsas G, et al. ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103(2):153-71.Full text  Abstract

13. Rehfeld JF, Gingras MH, Bardram L, et al. The Zollinger-Ellison syndrome and mismeasurement of gastrin. Gastroenterology. 2011 May;140(5):1444-53. Abstract

14. Metz DC. Diagnosis of the Zollinger-Ellison syndrome. Clin Gastroenterol Hepatol. 2012 Feb;10(2):126-30. Abstract

15. Rossi RE, Elvevi A, Citterio D, et al. Gastrinoma and Zollinger Ellison syndrome: a roadmap for the management between new and old therapies. World J Gastroenterol. 2021 Sep 21;27(35):5890-907.Full text  Abstract

16. Oh DS, Wang HS, Ohning GV, et al. Validation of a new endoscopic technique to assess acid output in Zollinger-Ellison syndrome. Clin Gastroenterol Hepatol. 2006 Dec;4(12):1467-73.Full text  Abstract

17. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

18. Goebel SU, Peghini PL, Goldsmith PK, et al. Expression of the calcium-sensing receptor in gastrinomas. J Clin Endocrinol Metab. 2000 Nov;85(11):4131-7. Abstract

19. Feng J, Petersen CD, Coy DH, et al. Calcium-sensing receptor is a physiologic multimodal chemosensor regulating gastric G-cell growth and gastrin secretion. Proc Natl Acad Sci U S A. 2010 Oct 12;107(41):17791-6.Full text  Abstract

20. Berna MJ, Hoffmann KM, Long SH, et al. 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 criteria, and correlations with clinical and tumoral features. Medicine (Baltimore). 2006 Nov;85(6):331-64.Full text  Abstract

21. Shibata C, Funayama Y, Fukushima K, et al. The glucagon provocative test for the diagnosis and treatment of Zollinger-Ellison syndrome. J Gastrointest Surg. 2008 Feb;12(2):344-9. Abstract

22. Stokkel MP, Rietbergen DD, Korse CM, et al. Somatostatin receptor scintigraphy and chromogranin A assay in staging and follow-up of patients with well-differentiated neuroendocrine tumors. Nucl Med Commun. 2011 Aug;32(8):731-7. Abstract

23. Krampitz GW, Norton JA. Current management of the Zollinger-Ellison syndrome. Adv Surg. 2013;47:59-79. Abstract

24. Ruf J, von Wedel F, Furth C, et al. Significance of a single-time-point somatostatin receptor SPECT/multiphase CT protocol in the diagnostic work-up of gastroenteropancreatic neuroendocrine neoplasms. J Nucl Med. 2016 Feb;57(2):180-5.Full text  Abstract

25. Wong KK, Gandhi A, Viglianti BL, et al. Endocrine radionuclide scintigraphy with fusion single photon emission computed tomography/computed tomography. World J Radiol. 2016 Jun 28;8(6):635-55.Full text  Abstract

26. Sainz-Esteban A, Olmos R, González-Sagrado M, et al. Contribution of ¹¹¹In-pentetreotide SPECT/CT imaging to conventional somatostatin receptor scintigraphy in the detection of neuroendocrine tumours. Nucl Med Commun. 2015 Mar;36(3):251-9. Abstract

27. Johnbeck CB, Knigge U, Kjær A. PET tracers for somatostatin receptor imaging of neuroendocrine tumors: current status and review of the literature. Future Oncol. 2014 Nov;10(14):2259-77.Full text  Abstract

28. Gabriel M, Decristoforo C, Kendler D, et al. 68Ga-DOTA-Tyr3-octreotide PET in neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and CT. J Nucl Med. 2007 Apr;48(4):508-18.Full text  Abstract

29. Wild D, Bomanji JB, Benkert P, et al. Comparison of 68Ga-DOTANOC and 68Ga-DOTATATE PET/CT within patients with gastroenteropancreatic neuroendocrine tumors. J Nucl Med. 2013 Mar;54(3):364-72.Full text  Abstract

30. Geijer H, Breimer LH. Somatostatin receptor PET/CT in neuroendocrine tumours: update on systematic review and meta-analysis. Eur J Nucl Med Mol Imaging. 2013 Oct;40(11):1770-80. Abstract

31. Wang J, Benhammou JN, Ghassemi K, et al. Endoscopic ultrasound-guided fine needle aspiration accurately diagnoses smaller pancreatic neuroendocrine tumors compared to computer tomography-guided fine needle aspiration. Gastroenterol Pancreatol Liver Disord. 2017;4(2):1-7.Full text

32. Thakker RV, Newey PJ, Walls GV, et al. Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1). J Clin Endocrinol Metab. 2012 Sep;97(9):2990-3011.Full text  Abstract

33. Kappelle WF, Valk GD, Leenders M, et al. Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study. Endoscopy. 2017 Jan;49(1):27-34. Abstract

34. ASGE Standards of Practice Committee, Coelho-Prabhu N, Forbes N, et al. Adverse events associated with EGD and EGD-related techniques. Gastrointest Endosc. 2022 Sep;96(3):389-401.e1.Full text  Abstract

35. Roy PK, Venzon DJ, Shojamanesh H, et al. Zollinger-Ellison syndrome: clinical presentation in 261 patients. Medicine (Baltimore). 2000 Nov;79(6):379-411. Abstract

36. Guzzo JL, Duncan M, Bass BL, et al. Severe and refractory peptic ulcer disease: the diagnostic dilemma: case report and comprehensive review. Dig Dis Sci. 2005 Nov;50(11):1999-2008. Abstract

37. Phan J, Benhammou JN, Pisegna JR. Gastric hypersecretory states: investigation and management. Curr Treat Options Gastroenterol. 2015 Dec;13(4):386-97.Full text  Abstract

38. Korman MG, Laver MC, Hansky J. Hypergastrinaemia in chronic renal failure. Br Med J. 1972 Jan 22;1(5794):209-10.Full text  Abstract

39. Taylor IL, Sells RA, McConnell RB, et al. Serum gastrin in patients with chronic renal failure. Gut. 1980 Dec;21(12):1062-7.Full text  Abstract

40. Toh BH. Pathophysiology and laboratory diagnosis of pernicious anemia. Immunol Res. 2017 Feb;65(1):326-30. Abstract

41. Storm AC, Fishman DS, et al. American Society for Gastrointestinal Endoscopy guideline on informed consent for GI endoscopic procedures. Gastrointest Endosc. 2022 Feb;95(2):207-15. Abstract

42. Forbes N, Coelho-Prabhu N, Al-Haddad MA, et al. Adverse events associated with EUS and EUS-guided procedures. Gastrointest Endosc. 2022 Jan;95(1):16-26. Abstract

43. Halfdanarson TR, Strosberg JR, Tang L, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and medical management of pancreatic neuroendocrine tumors. Pancreas. 2020 Aug;49(7):863-81. Abstract

44. Howe JR, Merchant NB, Conrad C, et al. The North American Neuroendocrine Tumor Society consensus paper on the surgical management of pancreatic neuroendocrine tumors. Pancreas. 2020 Jan;49(1):1-33.Full text  Abstract

45. Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text  Abstract

46. Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.Full text  Abstract

47. Pavel M, de Herder WW. ENETS Consensus guidelines for the standard of care in neuroendocrine tumors. Neuroendocrinology. 2017;105(3):193-5. Abstract

48. Sheen E, Triadafilopoulos G. Adverse effects of long-term proton pump inhibitor therapy. Dig Dis Sci. 2011 Apr;56(4):931-50. Abstract

49. Heidelbaugh JJ, Kim AH, Chang R, et al. Overutilization of proton-pump inhibitors: what the clinician needs to know. Therap Adv Gastroenterol. 2012 Jul;5(4):219-32.Full text  Abstract

50. Wilhelm SM, Rjater RG, Kale-Pradhan PB. Perils and pitfalls of long-term effects of proton pump inhibitors. Expert Rev Clin Pharmacol. 2013 Jul;6(4):443-51. Abstract

51. Willems RPJ, van Dijk K, Ket JCF, et al. Evaluation of the association between gastric acid suppression and risk of intestinal colonization with multidrug-resistant microorganisms: a systematic review and meta-analysis. JAMA Intern Med. 2020 Apr 1;180(4):561-71.Full text  Abstract

52. Yuan J, He Q, Nguyen LH, et al. Regular use of proton pump inhibitors and risk of type 2 diabetes: results from three prospective cohort studies. 2021 Jun;70(6):1070-7. Abstract

53. Moayyedi P, Eikelboom JW, Bosch J, et al. Safety of proton pump inhibitors based on a large, multi-year, randomized trial of patients receiving rivaroxaban or aspirin. Gastroenterology. 2019 Sep;157(3):682-691.e2.Full text  Abstract

54. Hansen KE, Nieves JW, Nudurupati S, et al. Dexlansoprazole and esomeprazole do not affect bone homeostasis in healthy postmenopausal women. Gastroenterology. 2019 Mar;156(4):926-934.e6.Full text  Abstract

55. Medicines and Healthcare products Regulatory Agency. Proton pump inhibitors: very low risk of subacute cutaneous lupus erythematosus. Sep 2015 [internet publication].Full text

56. Pisegna JR. Pharmacology of acid suppression in the hospital setting: focus on proton pump inhibition. Crit Care Med. 2002 Jun;30(6 Suppl):S356-61. Abstract

57. Scarpignato C, Gatta L, Zullo A, et al. Effective and safe proton pump inhibitor therapy in acid-related diseases - A position paper addressing benefits and potential harms of acid suppression. BMC Med. 2016 Nov 9;14(1):179.Full text  Abstract

58. Metz DC, Pisegna JR, Fishbeyn VA, et al. Currently used doses of omeprazole in Zollinger-Ellison syndrome are too high. Gastroenterology. 1992 Nov;103(5):1498-508. Abstract

59. Lew EA, Pisegna JR, Starr JA, et al. Intravenous pantoprazole rapidly controls gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. Gastroenterology. 2000 Apr;118(4):696-704. Abstract

60. Norton JA, Fraker DL, Alexander HR, Jensen RT. Value of surgery in patients with negative imaging and sporadic Zollinger-Ellison syndrome. Ann Surg. 2012 Sep;256(3):509-17.Full text  Abstract

61. Ito T, Igarashi H, Jensen RT. Zollinger-Ellison syndrome: recent advances and controversies. Curr Opin Gastroenterol. 2013 Nov;29(6):650-61.Full text  Abstract

62. Bartsch DK, Waldmann J, Fendrich V, et al. Impact of lymphadenectomy on survival after surgery for sporadic gastrinoma. Br J Surg. 2012 Sep;99(9):1234-40.Full text  Abstract

63. Sharzehi K, Sethi A, Savides T. AGA clinical practice update on management of subepithelial lesions encountered during routine endoscopy: expert review. Clin Gastroenterol Hepatol. 2022 Nov;20(11):2435-43.e4.Full text  Abstract

64. Norton JA, Fraker DL, Alexander HR, et al. Surgery increases survival in patients with gastrinoma. Ann Surg. 2006 Sep;244(3):410-9.Full text  Abstract

65. Imamura M, Komoto I, Ota S, et al. Biochemically curative surgery for gastrinoma in multiple endocrine neoplasia type 1 patients. World J Gastroenterol. 2011 Mar 14;17(10):1343-53.Full text  Abstract

66. Lopez CL, Waldmann J, Fendrich V, et al. Long-term results of surgery for pancreatic neuroendocrine neoplasms in patients with MEN1. Langenbecks Arch Surg. 2011 Dec;396(8):1187-96. Abstract

67. de Mestier L, Zappa M, Hentic O, et al. Liver transarterial embolizations in metastatic neuroendocrine tumors. Rev Endocr Metab Disord. 2017 Dec;18(4):459-71. Abstract

68. Yao JC, Phan A, Chang DZ, et al. Phase II study of RAD001 (everolimus) and depot octreotide (sandostatin LAR) in advanced low grade neuroendocrine carcinoma (LGNET). J Clin Oncol. 2007;25(suppl 16):4503.

69. Kulke M, Lenz HJ, Meropol NJ, et al. A phase 2 study to evaluate the efficacy and safety of SU11248 in patients (pts) with unresectable neuroendocrine tumors (NETs). J Clin Oncol. 2005;23(suppl):S4008.

70. Bushnell DL Jr, O'Dorisio TM, O'Dorisio MS, et al. 90Y-edotreotide for metastatic carcinoid refractory to octreotide. J Clin Oncol. 2010 Apr 1;28(10):1652-9.Full text  Abstract

71. Moertel CG, Lefkopoulo M, Lipsitz S, et al. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med. 1992 Feb 20;326(8):519-23.Full text  Abstract

72. Rivera E, Ajani JA. Doxorubicin, streptozocin, and 5-fluorouracil chemotherapy for patients with metastatic islet-cell carcinoma. Am J Clin Oncol. 1998 Feb;21(1):36-8. Abstract

73. Moertel CG, Lefkopoulo M, Lipsitz S, et al. The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med. 1994 Feb 15;120(4):302-9. Abstract

74. Cives M, Ghayouri M, Morse B, et al. Analysis of potential response predictors to capecitabine/temozolomide in metastatic pancreatic neuroendocrine tumors. Endocr Relat Cancer. 2016 Sep;23(9):759-67.Full text  Abstract

75. Kunz PL, Graham NT, Catalano PJ, et al. Randomized study of temozolomide or temozolomide and capecitabine in patients with advanced pancreatic neuroendocrine tumors (ECOG-ACRIN E2211). J Clin Oncol. 2023 Mar 1;41(7):1359-69.Full text  Abstract

76. Pisegna JR. Zollinger Ellison syndrome. Curr Treat Options Gastroenterol. 1999 Jun;2(3):195-204. Abstract

77. Moore AR, Boyce M, Steele IA, et al. Netazepide, a gastrin receptor antagonist, normalises tumour biomarkers and causes regression of type 1 gastric neuroendocrine tumours in a nonrandomised trial of patients with chronic atrophic gastritis. PLoS One. 2013 Oct 1;8(10):e76462.Full text  Abstract

78. Howe JR, Cardona K, Fraker DL, et al. The surgical management of small bowel neuroendocrine tumors: consensus guidelines of the North American Neuroendocrine Tumor Society. Pancreas. 2017 Jul;46(6):715-31.Full text  Abstract

79. Ito T, Igarashi H, Uehara H, et al. 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). 2013 May;92(3):135-81.Full text  Abstract

80. Weber HC, Venzon DJ, Lin JT, et al. Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. Gastroenterology. 1995 Jun;108(6):1637-49. Abstract

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