Skip to main content
PMC home page
Annals of Surgery logoLink to Annals of Surgery
. 1981 Oct;194(4):481–493. doi: 10.1097/00000658-198110000-00011

Pseudo-Zollinger-Ellison syndrome: hypergastrinemia, hyperchlorhydria without tumor.

S R Friesen, T Tomita
PMCID: PMC1345327  PMID: 7283508

Abstract

The purpose of the investigation was to detect ulcer patients having nontumorous hypergastrinemic hyperchlorhydria and to diagnostically differentiate this pseudo-Zollinger-Ellison syndrome from neurogenic duodenal ulcer disease and pancreatic gastrinomas. Nine patients having clinical, radiologic and humoral findings simulating the Zollinger-Ellison syndrome or severe duodenal ulcer disease were studied by physiologic provocative testing. The patients, not having pancreaticoduodenal gastrinomas, had an antral mucosal source of their moderate hypergastrinemia even after vagotomy with drainage, which was eliminated in eight patients treated by surgical antrectomy, resulting in normal serum gastrin concentrations. The pseudo-Zollinger-Ellison syndrome is, thus, characterized physiologically by an exaggerated gastrin response to meals, no response to secretin stimulation and pathologically by hyperfunctioning hyperplastic G cells of the antrum. The clinical, physiologic, pathologic and surgical features were integrated for accurate diagnosis and treatment.

Full text

PDF
485

Images in this article

482Fig. 1.
on p.482
483Fig. 2.
on p.483
484Fig. 3.
on p.484
488Fig. 7.
on p.488
489Fig. 8A.
on p.489
489Fig. 8B.
on p.489
490Fig. 8C.
on p.490

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Berson S. A., Yalow R. S. Radioimmunoassay in gastroenterology. Gastroenterology. 1972 May;62(5):1061–1084. [PubMed] [Google Scholar]
  2. Bhagavan B. S., Hofkin G. A., Woel G. M., Koss L. G. Zollinger-Ellison syndrome. Ultrastructural and histochemical observations in a child with endocrine tumorlets of gastric antrum. Arch Pathol. 1974 Oct;98(4):217–222. [PubMed] [Google Scholar]
  3. Bradley E. L., 3rd, Galambos J. T. Diagnosis of gastrinoma by the secretin suppression test. Surg Gynecol Obstet. 1976 Nov;143(5):784–788. [PubMed] [Google Scholar]
  4. Cowley D. J., Dymock I. W., Boyes B. E., Wilson R. Y., Stagg B. H., Lewin M. R., Polak J. M., Pearse A. G. Zollinger-Ellison syndrome type 1: clinical and pathological correlations in a case. Gut. 1973 Jan;14(1):25–29. doi: 10.1136/gut.14.1.25. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Deveney C. W., Deveney K. S., Jaffe B. M., Jones R. S., Way L. W. Use of calcium and secretin in the diagnosis of gastrinoma (Zollinger-Ellison syndrome). Ann Intern Med. 1977 Dec;87(6):680–686. doi: 10.7326/0003-4819-87-6-680. [DOI] [PubMed] [Google Scholar]
  6. Dunnick N. R., Doppman J. L., Mills S. R., McCarthy D. M. Computed tomographic detection of nonbeta pancreatic islet cell tumors. Radiology. 1980 Apr;135(1):117–120. doi: 10.1148/radiology.135.1.6244605. [DOI] [PubMed] [Google Scholar]
  7. Friesen S. R., Hermreck A. S., Brackett C. E., Schimke R. N. Case reports; Cimetidine in the management of synchronous crises of MEA I. World J Surg. 1980 Jan;4(1):123–129. doi: 10.1007/BF02393111. [DOI] [PubMed] [Google Scholar]
  8. Friesen S. R., Kimmel J. R., Tomita T. Pancreatic polypeptide as screening marker for pancreatic polypeptide apudomas in multiple endocrinopathies. Am J Surg. 1980 Jan;139(1):61–72. doi: 10.1016/0002-9610(80)90231-7. [DOI] [PubMed] [Google Scholar]
  9. Friesen S. R., McGuigan J. E. Ectopic apudocarcinomas and associated endocrine hyperplasias of the foregut. Ann Surg. 1975 Oct;182(4):371–385. doi: 10.1097/00000658-197510000-00003. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Friesen S. R., Schimke R. N., Pearse A. G. Genetic aspects of the Z-E syndrome: prospective studies in two kindred; antral gastrin cell hyperplasia. Ann Surg. 1972 Sep;176(3):370–383. doi: 10.1097/00000658-197209000-00013. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. GOODRICK W. I., DOCKERTY M. B. Zollinger Ellison syndromeassociated gastric tumor showing islet-cell features. Minn Med. 1963 Jul;46:634–639. [PubMed] [Google Scholar]
  12. Ganguli P. C., Polak J. M., Pearse A. G., Elder J. B., Hegarty M. Antral-gastrin-cell hyperplasia in peptic-ulcer disease. Lancet. 1974 Apr 6;1(7858):583–586. doi: 10.1016/s0140-6736(74)92647-6. [DOI] [PubMed] [Google Scholar]
  13. Giacobazzi P., Passaro E., Jr Preoperative angiography in the Zollinger-Ellison syndrome. Am J Surg. 1973 Jul;126(1):74–76. doi: 10.1016/s0002-9610(73)80098-4. [DOI] [PubMed] [Google Scholar]
  14. Goligher J. C., Feather D. B., Hall R., Hall R. A., Hopton D., Kenny T. E., Latchmore A. J., Matheson T., Shoesmith J. H., Smiddy F. G. Several standard elective operations for duodenal ulcer: ten to 16 year clinical results. Ann Surg. 1979 Jan;189(1):18–24. doi: 10.1097/00000658-197901000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Ingemansson S., Larsson L. I., Lunderquist A., Stadil F. Pancreatic vein catheterization with gastrin assay in normal patients and in patients with the Zollinger-Ellison syndrome. Am J Surg. 1977 Nov;134(5):558–563. doi: 10.1016/0002-9610(77)90433-0. [DOI] [PubMed] [Google Scholar]
  16. Keuppens F., Willems G., De Graef J., Woussen-Colle M. C. Antral gastrin cell hyperplasia in patients with peptic ulcer. Ann Surg. 1980 Mar;191(3):276–281. doi: 10.1097/00000658-198003000-00004. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Kimmel J. R., Hayden L. J., Pollock H. G. Isolation and characterization of a new pancreatic polypeptide hormone. J Biol Chem. 1975 Dec 25;250(24):9369–9376. [PubMed] [Google Scholar]
  18. Korman M. G., Laver M. C., Hansky J. Hypergastrinaemia in chronic renal failure. Br Med J. 1972 Jan 22;1(5794):209–210. doi: 10.1136/bmj.1.5794.209. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Korman M. G., Scott D. F., Hansky J., Wilson H. Hypergastrinaemia due to an excluded gastric antrum: a proposed method for differentiation from the Zollinger ellison syndrome. Aust N Z J Med. 1972 Aug;2(3):266–271. doi: 10.1111/j.1445-5994.1972.tb03073.x. [DOI] [PubMed] [Google Scholar]
  20. Korman M. G., Soveny C., Hansky J. Serum gastrin in duodenal ulcer. I. Basal levels and effect of food and atropine. Gut. 1971 Nov;12(11):899–902. doi: 10.1136/gut.12.11.899. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Lamers C. B., Diemel J. M., Roeffen W. Pancreatic polypeptide: A marker for Zollinger-Ellison syndrome? Lancet. 1978 Aug 5;2(8084):326–326. doi: 10.1016/s0140-6736(78)91736-1. [DOI] [PubMed] [Google Scholar]
  22. Larsson L. I., Ljungberg O., Sundler F., Håkanson R., Svensson S. O., Rehfeld J., Stadil R., Holst J. Antor-pyloric gastrinoma associated with pancreatic nesidioblastosis and proliferation of islets. Virchows Arch A Pathol Pathol Anat. 1973;360(4):305–314. doi: 10.1007/BF00548351. [DOI] [PubMed] [Google Scholar]
  23. McCarthy D. M. The place of surgery in the Zollinger-Ellison syndrome. N Engl J Med. 1980 Jun 12;302(24):1344–1347. doi: 10.1056/NEJM198006123022404. [DOI] [PubMed] [Google Scholar]
  24. Passaro E., Jr, Basso N., Walsh J. H. Calcium challenge in the Zollinger-Ellison syndrome. Surgery. 1972 Jul;72(1):60–67. [PubMed] [Google Scholar]
  25. Pearse A. G., Bussolati G. Immunofluorescence studies on the distribution of gastrin cells in different clinical states. Gut. 1970 Aug;11(8):646–648. doi: 10.1136/gut.11.8.646. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Polak J. M., Bloom S. R., Adrian T. E., Heitz P., Bryant M. G., Pearse A. G. Pancreatic polypeptide in insulinomas, gastrinomas, vipomas, and glucagonomas. Lancet. 1976 Feb 14;1(7955):328–330. doi: 10.1016/s0140-6736(76)90084-2. [DOI] [PubMed] [Google Scholar]
  27. Polak J. M., Bloom S. R., Bishop A. E., McCrossan M. V. D cell pathology in duodenal ulcers and achlorhydria. Metabolism. 1978 Sep;27(9 Suppl 1):1239–1242. doi: 10.1016/0026-0495(78)90051-3. [DOI] [PubMed] [Google Scholar]
  28. Polak J. M., Bussolati G., Pearse A. G. Cytochemical, immunofluorescence and ultrastructural investigations on the antral G cells in hyperparathyroidism. Virchows Arch B Cell Pathol. 1971;9(3):187–197. doi: 10.1007/BF02894045. [DOI] [PubMed] [Google Scholar]
  29. Polak J. M., Stagg B., Pearse A. G. Two types of Zollinger-Ellison syndrome: immunofluorescent, cytochemical and ultrastructural studies of the antral and pancreatic gastrin cells in different clinical states. Gut. 1972 Jul;13(7):501–512. doi: 10.1136/gut.13.7.501. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Royston C. M., Brew D. S., Garnham J. R., Stagg B. H., Polak J. The Zollinger-Ellison syndrome due to an infiltrating tumour of the stomach. Gut. 1972 Aug;13(8):638–642. doi: 10.1136/gut.13.8.638. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Russo A., Buffa R., Grasso G., Giannone G., Sanfilippo G., Sessa F., Solcia E. Gastric gastrinoma and diffuse G cell hyperplasia associated with chronic atrophic gastritis. Endoscopic detection and removal. Digestion. 1980;20(6):416–419. doi: 10.1159/000198484. [DOI] [PubMed] [Google Scholar]
  32. Straus E., Gerson C. D., Yalow R. S. Hypersecretion of gastrin associated with the short bowel syndrome. Gastroenterology. 1974 Feb;66(2):175–180. [PubMed] [Google Scholar]
  33. Straus E., Greenstein R. J., Yalow R. S. Plasma-secretin in management of cimetidine therapy for Zollinger-Ellison syndrome. Lancet. 1978 Jul 8;2(8080):73–75. doi: 10.1016/s0140-6736(78)91385-5. [DOI] [PubMed] [Google Scholar]
  34. Stremple J. F., Watson C. G. Serum calcium, serum gastrin, and gastric acid secretion before and after parathyroidectomy for hyperparathyroidism. Surgery. 1974 Jun;75(6):841–852. [PubMed] [Google Scholar]
  35. Taylor C. R. Immunoperoxidase techniques: practical and theoretical aspects. Arch Pathol Lab Med. 1978 Mar;102(3):113–121. [PubMed] [Google Scholar]
  36. Wilson R. Y., Boyes B. E., Stagg B. H., Lewin M. R., Polak J. M., Pearse A. G., Dymock I. W., Cowley D. J. Antral G-cell hyperplasia with hypergastrinaemia producing a Zollinger-Ellison syndrome. Gut. 1972 Oct;13(10):848–849. [PubMed] [Google Scholar]
  37. ZOLLINGER R. M., ELLISON E. H. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955 Oct;142(4):709-23; discussion, 724-8. [PMC free article] [PubMed] [Google Scholar]
  38. Zollinger R. M., Ellison E. C., Fabri P. J., Johnson J., Sparks J., Carey L. C. Primary peptic ulcerations of the jejunum associated with islet cell tumors. Twenty-five-year appraisal. Ann Surg. 1980 Sep;192(3):422–430. doi: 10.1097/00000658-198009000-00018. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Annals of Surgery are provided here courtesy of Lippincott, Williams, and Wilkins

RESOURCES