Zollinger-Ellison Syndrome: An Uncommon Cause of Peptic Ulcer Disease and Chronic Diarrhea

Authors

  • Thagoon Kanjanopas Department of Internal Medicine, Hatyai Hospital, Songkhla, Thailand
  • Arunchai Chang Division of Gastroenterology, Department of Internal Medicine, Hatyai Hospital, Songkhla, Thailand
  • Keerati Akarapatima Division of Gastroenterology, Department of Internal Medicine, Hatyai Hospital, Songkhla, Thailand
  • Cheep Charoenlap Department of Anatomical Pathology, Hatyai Hospital, Songkhla, Thailand
  • Songklod Pakdeejit Division of Intervention Radiology, Department of Radiology, Hatyai Hospital, Songkhla, Thailand
  • Attapon Rattanasupa Division of Gastroenterology, Department of Internal Medicine, Hatyai Hospital, Songkhla, Thailand

Keywords:

Zollinger-Ellison syndrome, Gastrinoma, Chronic diarrhea, Peptic ulcer disease, Multiple Endocrine Neoplasia-type 1

Abstract

Background and Objective: Zollinger-Ellison syndrome (ZES) is an extremely rare condition caused by a neuroendocrine tumor, namely gastrinoma, associated with gastric acid overproduction. The typical presentations include recurrent peptic ulcers accompanied by chronic diarrhea.

Method: We present a case report using the information extracted from medical records at Hatyai Hospital.

Results: A 55-year-old woman presented with intermittent epigastric pain and chronic diarrhea. Upper endoscopy revealed multiple small gastric polyps accompanied by multiple ulcers in the postbulbar region. Magnetic resonance imaging showed a hypervascular tumor located in the gastrinoma triangle with liver metastasis. The condition was diagnosed as an aggressive gastrinoma based on the histopathology. The patient’s symptoms responded well to supportive treatment.

Conclusion: The present case emphasizes the importance of having high clinical suspicion of ZES, especially in patients presenting with peptic ulcer disease and chronic diarrhea, which may help in the early diagnosis of the patient and provide appropriate treatment.

References

Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955;142:709-23. discussion, 24-8.

Gregory RA, Tracy HJ, French JM, Sircus W. Extraction of a gastrin-like substance from a pancreatic tumour in a case of Zollinger-Ellison syndrome. Lancet 1960;1:1045-8.

Mendelson AH, Donowitz M. Catching the Zebra: Clinical Pearls and Pitfalls for the Successful Diagnosis of Zollinger-Ellison Syndrome. Dig Dis Sci 2017;62:2258-65.

Ellison EC, Sparks J, Verducci JS, Johnson JA, Muscarella P, Bloomston M, et al. 50-year appraisal of gastrinoma: recommendations for staging and treatment. J Am Coll Surg 2006;202:897-905.

Roy PK, Venzon DJ, Shojamanesh H, Abou-Saif A, Peghini P, Doppman JL, et al. Zollinger-Ellison syndrome. Clinical presentation in 261 patients. Medicine (Baltimore) 2000;79:379-411.

Yao JC, Eisner MP, Leary C, Dagohoy C, Phan A, Rashid A, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol 2007;14:3492-500.

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 cases from the literature. Medicine (Baltimore) 2004;83:43-83.

Epelboym I, Mazeh H. Zollinger-Ellison syndrome: classical considerations and current controversies. Oncologist 2014;19:44-50.

Atri D, Furfaro D, Dhaliwal G, Feingold KR, Manesh R. Going from A to Z. N Engl J Med 2018;378:73-9.

Ito T, Igarashi H, Jensen RT. Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances. Best Pract Res Clin Gastroenterol 2012;26:737-53.

Lipiński M, Rydzewska G, Foltyn W, Andrysiak-Mamos E, Bałdys-Waligórska A, Bednarczuk T, et al. Gastroduodenal neuroendocrine neoplasms, including gastrinoma - management guidelines (recommended by the Polish Network of Neuroendocrine Tumours). Endokrynol Pol 2017;68:138-53.

Jalving M, Koornstra JJ, Wesseling J, Boezen HM, S DEJ, Kleibeuker JH. Increased risk of fundic gland polyps during long-term proton pump inhibitor therapy. Aliment Pharmacol Ther 2006;24:1341-8.

Modlin IM, Gilligan CJ, Lawton GP, Tang LH, West AB, Darr U. Gastric carcinoids. The Yale Experience. Arch Surg 1995;130:250-5; discussion 5-6.

Berna MJ, Hoffmann KM, Serrano J, Gibril F, Jensen RT. Serum gastrin in Zollinger-Ellison syndrome: I. Prospective study of fasting serum gastrin in 309 patients from the National Institutes of Health and comparison with 2229 cases from the literature. Medicine (Baltimore) 2006;85:295-330.

Stabile BE, Morrow DJ, Passaro E, Jr. The gastrinoma triangle: operative implications. Am J Surg 1984;147:25-31.

Anlauf M, Garbrecht N, Henopp T, Schmitt A, Schlenger R, Raffel A, et al. Sporadic versus hereditary gastrinomas of the duodenum and pancreas: distinct clinico-pathological and epidemiological features. World J Gastroenterol 2006;12:5440-6.

Tomassetti P, Campana D, Piscitelli L, Mazzotta E, Brocchi E, Pezzilli R, et al. Treatment of Zollinger-Ellison syndrome. World J Gastroenterol 2005;11:5423-32.

Lee L, Ito T, Jensen RT. Imaging of pancreatic neuroendocrine tumors: recent advances, current status, and controversies. Expert Rev Anticancer Ther 2018;18:837-60.

Ito T, Lee L, Jensen RT. Treatment of symptomatic neuroendocrine tumor syndromes: recent advances and controversies. Expert Opin Pharmacother 2016;17:2191-205.

Dromain C, Déandréis D, Scoazec JY, Goere D, Ducreux M, Baudin E, et al. Imaging of neuroendocrine tumors of the pancreas. Diagn Interv Imaging 2016;97:1241-57.

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

Jin XF, Spampatti MP, Spitzweg C, Auernhammer CJ. Supportive therapy in gastroenteropancreatic neuroendocrine tumors: Often forgotten but important. Rev Endocr Metab Disord 2018;19:145-58.

Falconi M, Eriksson B, Kaltsas G, Bartsch DK, Capdevila J, Caplin M, et al. ENETS Consensus Guidelines Update for the Management of Patients with Functional Pancreatic Neuroendocrine Tumors and Non-Functional Pancreatic Neuroendocrine Tumors. Neuroendocrinology 2016;103:153-71.

Jensen RT, Berna MJ, Bingham DB, Norton JA. Inherited pancreatic endocrine tumor syndromes: advances in molecular pathogenesis, diagnosis, management, and controversies. Cancer 2008;113:1807-43.

Pape UF, Jann H, Müller-Nordhorn J, Bockelbrink A, Berndt U, Willich SN, et al. Prognostic relevance of a novel TNM classification system for upper gastroenteropancreatic neuroendocrine tumors. Cancer 2008;113:256-65.

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Published

2022-06-24

How to Cite

1.
Kanjanopas T, Chang A, Akarapatima K, Charoenlap C, Pakdeejit S, Rattanasupa A. Zollinger-Ellison Syndrome: An Uncommon Cause of Peptic Ulcer Disease and Chronic Diarrhea. SRIMEDJ [Internet]. 2022 Jun. 24 [cited 2024 Dec. 27];37(3):287-92. Available from: https://li01.tci-thaijo.org/index.php/SRIMEDJ/article/view/253140

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Case report