Zollinger-Ellison syndrome revisited: Diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion

Current Gastroenterology Reports

Volumn 6, Issue 6, 2004, Pages 454-463

  Gibril, Fathia ^a   Jensen, Robert T ^a  

^a NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BIOLOGICAL MARKER; CHORIONIC GONADOTROPIN ALPHA SUBUNIT; CHORIONIC GONADOTROPIN BETA SUBUNIT; CHROMOGRANIN A; CHROMOGRANIN B; CIMETIDINE; FAMOTIDINE; GASTRIN; HISTAMINE; HISTAMINE H1 RECEPTOR ANTAGONIST; HISTAMINE H2 RECEPTOR ANTAGONIST; NEURON SPECIFIC ENOLASE; OMEPRAZOLE; PANCREASTATIN; PANTOPRAZOLE; PROTON PUMP INHIBITOR; RANITIDINE; SEROTONIN; TUMOR MARKER;

CARCINOID; CLINICAL FEATURE; CYANOCOBALAMIN DEFICIENCY; DIAGNOSTIC ERROR; DIAGNOSTIC VALUE; DIET RESTRICTION; DISEASE ASSOCIATION; DOSE CALCULATION; DRUG EFFICACY; DRUG MEGADOSE; DRUG SAFETY; ENZYME BLOOD LEVEL; GASTRIN BLOOD LEVEL; GASTRINOMA; GASTROSCOPY; GENETIC DISORDER; GONADOTROPIN BLOOD LEVEL; HISTAMINE BLOOD LEVEL; HUMAN; HYPERGASTRINEMIA; LONG TERM CARE; MULTIPLE ENDOCRINE NEOPLASIA; NON INVASIVE MEASUREMENT; NONHUMAN; PROGNOSIS; PROTEIN BLOOD LEVEL; REVIEW; SENSITIVITY ANALYSIS; SEROTONIN BLOOD LEVEL; SIDE EFFECT; STOMACH ACID SECRETION; STOMACH PH; STOMACH SCINTISCANNING; STOMACH SURGERY; TACHYPHYLAXIS; TUMOR GROWTH; ZOLLINGER ELLISON SYNDROME;

EID: 9944241273     PISSN: 15228037     EISSN: None     Source Type: Journal    
DOI: 10.1007/s11894-004-0067-5     Document Type: Review

References (71)

1. Jensen RT: Gastrinoma. Bailliere's Clin Gastroenterol 1996, 10:555-766.
2. Roy P, Venzon DJ, Shojamanesh H, et al.: Zollinger-Ellison syndrome: clinical presentation in 261 patients. Medicine 2000, 79:379-411.
3. Delle Fave GF, Frati L, Jensen RT: Advances in digestive endocrine tumours. Ital J Gastroenterol Hepatol 1999, 31: 93-S230.
4. Corleto VD, Delle Fave G, Jensen RT: Molecular insights into gastrointestinal neuroendocrine tumors: importance and recent advances. Dig Liver Dis 2002, 34:668-680.
5. 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.
6. Norton JA, Jensen RT: Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome. Ann Surg 2004, in press.
7. Jensen RT: Carcinoid and pancreatic endocrine tumors: recent advances in molecular pathogenesis, localization, and treatment Curr Opin Oncol 2000, 12:368-377.
8. Corleto VD, 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, 15:1555-1561.
9. Jensen RT, Gardner JD: Gastrinoma. In The Pancreas: Biology, Pathobiology and Disease. Edited by Go VLW, DiMagno EP, Gardner JD, et al. New York: Raven Press; 1993:931-978.
10. Jensen RT: Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1. J Intern Med 1998, 243:477-488.
11. Gibril F, Schumann M, Pace A, Jensen RT: Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective study of 107 cases and comparison with 1009 patients from the literature Medicine 2004, 83:43-83.
12. Weber HC, Venzon DJ, Jensen RT, Metz DC: Studies on the interrelation between Zollinger-Ellison syndrome, Helicobacter pylori and proton pump inhibitor therapy. Gastroenterology 1997, 112: 4-91.
13. Jensen RT: Use of omeprazole and other proton pump inhibitors in the Zollinger-Ellison syndrome. In Milestones in Drug Therapy. Edited by Olbe L. Basel, Switzerland: Birkhauser Verlag AG; 1999:205-221.
14. Jensen RT: Zollinger-Ellison syndrome. In Surgical Endocrinology: Clinical Syndromes. Edited by Doherty GM, Skogseid B. Philadelphia: Lippincott Williams & Wilkins; 2001:291-344.
15. 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.
16. Miller LS, Vinayek R, Frucht H, et al.: Reflux esophagitis in patients with Zollinger-Ellison syndrome. Gastroenterology 1990, 98:341-346.
17. Hirschowitz BI, Simmons J, Mohnen J: Long-term lansoprazole control of gastric acid and pepsin secretion in ZE and non-ZE hypersecretors: a prospective 10-year study. Aliment Pharmacol Ther 2001, 15:1795-1806.
18. Metz DC, Jensen RT: Advances in gastric antisecretory therapy in Zollinger-Ellison syndrome. In Endocrine Tumors of the Pancreas: Recent Advances in Research and Management. Series: Frontiers of Gastrointestinal Research. Edited by Mignon M, Jensen RT. Basel, Switzerland: S. Karger; 1995:240-257.
19. Jensen RT: Gastrinoma as a model for prolonged hypergastrinemia in man. In Gastrin. Edited by Walsh JH. New York: Raven Press; 1993:373-393.
20. Chiang HC, O'Dorisio TM, Huang SC, et al.: Multiple hormone elevations in patients with Zollinger-Ellison syndrome: prospective study of clinical significance and of the development of a second symptomatic pancreatic endocrine tumor syndrome. Gastroenterology 1990, 99:1565-1575.
21. Abou-Saif A, Gibril F, Ojeaburu JV, et al.: Prospective study of the ability of serial measurements of serum chromogranin A and gastrin to detect changes in tumor burden in patients with gastrinomas. Cancer 2003, 98:249-261.
22. Rehfeld JF, Bardram L: Prohormone processing and pancreatic endocrine tumors. In Endocrine Tumors of the Pancreas: Recent Advances in Research and Management. Series: Frontiers of Gastrointestinal Research. Edited by Mignon M, Jensen RT. Basel, Switzerland: S. Karger; 1995:84-98.
23. Rehfeld JF, van Solinge WW: The tumor biology of gastrin and cholecystokinin. Adv Cancer Res 1994, 63:295-347.
24. Bardram L: Progastrin in pancreas and the Zollinger-Ellison syndrome, Scand J Gastroenterol 1990, 25: 185-1195.
25. Bardram L: Progastrin in serum from Zollinger-Ellison patients: an indicator of malignancy? Gastroenterology 1990, 98:1420-1426.
26. Oberg K, Janson ET, Eriksson B: Tumour markers in neuroendocrine tumours. Ital J Gastroenterol Hepatol 1999, 31:S160-S162.
27. Bardram L, Agner T, Hagen C: Levels of alpha subunits of gonadotropins can be increased in Zollinger-Ellison syndrome, both in patients with malignant tumors and apparently benign disease. Acta Endocrinol (Copenh) 1988, 118:135-141.
28. Baudin E, Gigliotti A, Ducreux M, et al.: Neuron-specific enolase and chromogranin A as markers of neuroendocrine tumours. Br J Cancer 1998, 78:1102-1107.
29. Syversen U, Mignon M, Bonfils S, et al.: Chromogranin A and pancreastatin-like immunoreactivity in serum of gastrinoma patients. Acta Oncol 1993, 32:161-165.
30. Nobels FR, Kwekkeboom DJ, Coopmans W, et al.: Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones. J Clin Endocrinol Metab 1997, 82:2622-2628.
31. Goebel SU, Serrano J, Yu F, et al.: Prospective study of the value of serum chromogranin A or serum gastrin levels in assessment of the presence, extent, or growth of gastrinomas. Cancer 1999, 85:1470-1483.
32. Taupenot L, Harper KL, O'Connor DT: The chromogranin-secretogranin family. N Engl J Med 2003, 348: 134-1149.
33. Norton JA, Cornelius MJ, Doppman JL, et al.: Effect of parathyroidectomy in patients with hyperparathyroidism, Zollinger-Ellison syndrome and multiple endocrine neoplasia Type I: a prospective study. Surgery 1987, 102:958-966.
34. Fishbeyn VA, Norton JA, Benya RV, et al.: Assessment and prediction of long-term cure in patients with Zollinger-Ellison syndrome: the best approach. Ann Intern Med 1993, 119: 99-206.
35. Roy P, Venzon DJ, Feigenbaum KM, et al.: 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.
36. Frucht H, Howard JM, Slaff JI, et al.: Secretin and calcium provocative tests in the Zollinger-Ellison syndrome: a prospective study. Ann Intern Med 1989, 111:713-722.
37. Peracchi M, Conte D, Gebbia C, et al.: Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1. Eur J Epidemiol 2003, 148:39-43.
38. Panzuto F, Severi C, Cannizzaro R, et al.: Utility of combined use of plasma levels of chromogranin A and pancreatic polypeptide in the diagnosis of gastrointestinal and pancreatic endocrine tumors. J Endocrinol Invest 2004, 27:6-11.
39. Peghini PL, Annibale B, Azzoni C, et al.: Effect of chronic hypergastrinemia on human enterochromaffin-like cells: insights from patients with sporadic gastrinomas. Gastroenterology 2002, 123:68-85.
40. Stridsberg M, Eriksson B, Oberg K, Janson ET: A comparison between three commercial kits for chromogranin A measurements. J Endocrinol 2003, 177:337-341.
41. Bajetta E, Ferrari L, Martinetti A, et al.: Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer 1999, 86:858-865.
42. Berger AC, Gibril F, Venzon DJ, et al.: Prognostic value of initial fasting serum gastrin level in patients with Zollinger-Ellison syndrome. J Clin Oncol 2001, 19: 051-3057.
43. Grossmann M, Trautmann ME, Poertl S, et al.: Alpha-subunit and human chorionic gonadotropin-beta immunoreactivity in patients with malignant endocrine gastroenteropancreatic tumours. Eur J Clin Invest 1994, 24:131-136.
44. Bashir S, Gibril F, Ojeaburu J, et al.: Prospective study of the ability of histamine, serotonin or chromogranin A levels to identify gastric carcinoids in patients with gastrinomas. Aliment Pharmacol Ther 2002, 16:1367-1382.
45. Rindi G, Bordi C, Rappel S, et al.: Gastric carcinoids and neuroendocrine carcinomas: pathogenesis, pathology, and behavior. World J Surg 1996, 20:168-172.
46. Waldum HL, Fossmark R, Bakke I, et al.: Hypergastrinemia in animals and man: causes and consequences. Scand J Gastroenterol 2004, 39:505-509.
47. Solcia E, Fiocca R, Villani L, et al.: Hyperplastic, dysplastic, and neoplastic enterochromaffin-like-cell proliferations of the gastric mucosa: classification and histogenesis. Am J Surg Pathol 1995, 19:S1-S7.
48. Norton JA, Fraker DL, Alexander HR, et al.: Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med 1999, 341:635-644.
49. Cadiot G, Lehy T, Ruszniewski P, et al.: Gastric endocrine cell evolution in patients with Zollinger-Ellison syndrome: influence of gastrinoma growth and long-term omeprazole treatment. Dig Dis Sci 1993, 38:1307-1317.
50. Lehy T, Mignon M, Cadiot G, et al.: Gastric endocrine cell behavior in Zollinger-Ellison patients upon long-term potent antisecretory treatment. Gastroenterology 1989, 96: 1029-1040.
51. Norton JA, Melcher M, Gibril F, Jensen RT: Gastric carcinoid in Multiple Endocrine Neoplasia Type 1 (MEN1) patients with Zollinger-Ellison syndrome can be symptomatic, demonstrate aggressive growth and require surgery. Surgery 2004, in press.
52. Gibril F, Reynolds JC, Roy PK, et al.: Ability of somatostatin receptor scintigraphy to identify patients with localized gastric carcinoids: a prospective study. J Nucl Med 2000, 41: 646-1656.
53. Gibril F, Reynolds JC, Chen CC, et al.: Specificity of somatostatin receptor scintigraphy: a prospective study and the effects of false positive localizations on management in patients with gastrinomas. J Nucl Med 1999, 40:539-553.
54. Jensen RT: Pancreatic endocrine tumors. In Harrison's Principles of Internal Medicine. Edited by Braunwald E, Fauci AS, Kaspar DL, et al. New York: McGraw-Hill, Medical Publication Division; 2001:593-603.
55. Thakker RV: Multiple endocrine neoplasia type 1. Endocrinol Metab Clin North Am 2000, 29:541-567.
56. Marx S, Spiegel AM, Skarulis MC, et al.: Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med 1998, 129:484-494.
57. Metz DC, Strader DB, Orbuch M, et al.: Use of omeprazole in Zollinger-Ellison: a prospective nine-year study of efficacy and safety. Aliment Pharmacol Ther 1993, 7: 97-610.
58. Gibril F, Chen Y-J, Schrump DS, et al.: Prospective study of thymic carcinoids in patients with multiple endocrine neoplasia type 1. J Clin Endocrinol Metab 2003, 88:1066-1081.
59. Benya RV, Metz DC, Venzon DL, et al.: Zollinger-Ellison syndrome can be the initial endocrine manifestation in patients with multiple endocrine neoplasia type 1. Am J Med 1994, 97:436-444.
60. Waxman I, Gardner JD, Jensen RT, Maton PN: Peptic ulcer perforation as the presentation of Zollinger-Ellison syndrome. Dig Dis Sci 1991, 16:19-24.
61. Jensen RT: Overview of chronic diarrhea caused by functional neuroendocrine neoplasms. Semin Gastrointest Dis 1999, 10:156-172.
62. Strader DB, Benjamin S, Orbuch M, et al.: Esophageal function and occurrence of Barrett's esophagus in Zollinger-Ellison syndrome. Digestion 1995, 56:347-356.
63. Rambaud JC, Hautefeuille M, Ruskone A, Jacquenod P: Diarrhoea due to circulating agents. Clinics in GI 1986, 15: 03-629.
64. Jensen RT. Treatment of pancreatic Zollinger-Ellison syndrome and other gastric hypersecretory states. In Therapy of Digestive Disorders. A Companion to Sleisenger and Fordtran's Gastrointestinal and Liver Diseases. Edited by Wolfe MM, Philadelphia: WB Saunders; 2000:169-187.
65. Raufman JP, Collins SM, Pandol SJ, et al.: Reliability of symptoms in assessing control of gastric acid secretion in patients with Zollinger-Ellison syndrome. Gastroenterology 1983, 84:108-113.
66. Termanini B, Gibril F, Stewart CA, et al.: A prospective study of the effectiveness of low dose omeprazole as initial therapy in Zollinger-Ellison syndrome. Aliment Pharmacol Ther 1996, 10:61-71.
67. Lew EA, Pisegna JR, Starr JA, et al.: Intravenous pantoprazole rapidly controls gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. Gastroenterology 2000, 118: 96-704.
68. Saeed ZA, Norton JA, Frank WO, et al.: Parenteral antisecretory drug therapy in patients with Zollinger-Ellison syndrome Gastroenterology 1989, 96:1393-1402.
69. Vinayek R, Hahne WF, Euler AR, et al.: Parenteral control of gastric hypersecretion in patients with Zollinger-Ellison syndrome. Dig Dis Sci 1993, 38:1857-1865.
70. Termanini B, Gibril F, Sutliff VE, III, et al.: Effect of long-term gastric acid suppressive therapy on serum vitamin B12 levels in patients with Zollinger-Ellison syndrome. Am J Med 1998, 104:422-430.
71. Stewart CA, Termanini B, Sutliff VE, et al.: Assessment of the risk of iron malabsorption in patients with Zollinger-Ellison syndrome treated with long-term gastric acid antisecretory therapy. Aliment Pharmacol Ther 1998, 12: 3-98.