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Zollinger-Ellison syndrome. Current concepts in diagnosis and management.
N Engl J Med. 1987 Nov 05; 317(19):1200-9.NEJM

Abstract

The Zollinger-Ellison syndrome, although uncommon, is not rare, and most patients with the disorder present with clinical manifestations similar to those of patients with common peptic ulcer. Early studies emphasized death due to complications of massive gastric acid hypersecretion. However, with the availability of potent antisecretory agents to control acid secretion, death is now more frequently associated with the metastatic potential of slowly growing but malignant gastrinomas. Therefore, physicians should maintain a high degree of suspicion of the Zollinger-Ellison syndrome in assessing patients with either chronic peptic ulcer or unexplained secretory diarrhea. An evaluation aimed at early diagnosis of the Zollinger-Ellison syndrome should be instituted in such patients and should begin with a determination of the fasting serum gastrin level. At least 50 percent of patients with gastrinoma will have nondiagnostic serum gastrin concentrations and will therefore require provocative testing to establish the correct diagnosis. After the presence of the syndrome is established, patients should be treated with a potent antisecretory agent in doses sufficient to reduce basal acid output to less than 10 mmol in the hour preceding administration of the next dose. Although some patients may be maintained satisfactorily in this manner for extended periods, an approach aimed at tumor localization and extirpation is recommended in most patients. Preoperative evaluation should begin with CT scanning with intravenous contrast material. Selective angiography, and occasionally, portal venous sampling for gastrin, should be performed if the location and extent of tumor remain in question. If metastatic disease is demonstrated, or if MEN-I is present, surgery aimed at tumor resection, although it is occasionally effective, will probably be unsuccessful. Because of the considerable morbidity and mortality associated with pancreatoduodenectomy, it should not be performed for unresectable tumor in the head of the pancreas. In other patients with the Zollinger-Ellison syndrome, exploratory surgery should be performed; this should include a careful search for, and resection of, all pancreatic and extrapancreatic gastrinomas. With this approach, it is likely that at least 20 percent of all patients with the Zollinger-Ellison syndrome can be cured.

Authors+Show Affiliations

Harvard Digestive Diseases Center, Beth Israel Hospital, Boston, MA.No affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

3309661

Citation

Wolfe, M M., and R T. Jensen. "Zollinger-Ellison Syndrome. Current Concepts in Diagnosis and Management." The New England Journal of Medicine, vol. 317, no. 19, 1987, pp. 1200-9.
Wolfe MM, Jensen RT. Zollinger-Ellison syndrome. Current concepts in diagnosis and management. N Engl J Med. 1987;317(19):1200-9.
Wolfe, M. M., & Jensen, R. T. (1987). Zollinger-Ellison syndrome. Current concepts in diagnosis and management. The New England Journal of Medicine, 317(19), 1200-9.
Wolfe MM, Jensen RT. Zollinger-Ellison Syndrome. Current Concepts in Diagnosis and Management. N Engl J Med. 1987 Nov 5;317(19):1200-9. PubMed PMID: 3309661.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Zollinger-Ellison syndrome. Current concepts in diagnosis and management. AU - Wolfe,M M, AU - Jensen,R T, PY - 1987/11/5/pubmed PY - 1987/11/5/medline PY - 1987/11/5/entrez SP - 1200 EP - 9 JF - The New England journal of medicine JO - N Engl J Med VL - 317 IS - 19 N2 - The Zollinger-Ellison syndrome, although uncommon, is not rare, and most patients with the disorder present with clinical manifestations similar to those of patients with common peptic ulcer. Early studies emphasized death due to complications of massive gastric acid hypersecretion. However, with the availability of potent antisecretory agents to control acid secretion, death is now more frequently associated with the metastatic potential of slowly growing but malignant gastrinomas. Therefore, physicians should maintain a high degree of suspicion of the Zollinger-Ellison syndrome in assessing patients with either chronic peptic ulcer or unexplained secretory diarrhea. An evaluation aimed at early diagnosis of the Zollinger-Ellison syndrome should be instituted in such patients and should begin with a determination of the fasting serum gastrin level. At least 50 percent of patients with gastrinoma will have nondiagnostic serum gastrin concentrations and will therefore require provocative testing to establish the correct diagnosis. After the presence of the syndrome is established, patients should be treated with a potent antisecretory agent in doses sufficient to reduce basal acid output to less than 10 mmol in the hour preceding administration of the next dose. Although some patients may be maintained satisfactorily in this manner for extended periods, an approach aimed at tumor localization and extirpation is recommended in most patients. Preoperative evaluation should begin with CT scanning with intravenous contrast material. Selective angiography, and occasionally, portal venous sampling for gastrin, should be performed if the location and extent of tumor remain in question. If metastatic disease is demonstrated, or if MEN-I is present, surgery aimed at tumor resection, although it is occasionally effective, will probably be unsuccessful. Because of the considerable morbidity and mortality associated with pancreatoduodenectomy, it should not be performed for unresectable tumor in the head of the pancreas. In other patients with the Zollinger-Ellison syndrome, exploratory surgery should be performed; this should include a careful search for, and resection of, all pancreatic and extrapancreatic gastrinomas. With this approach, it is likely that at least 20 percent of all patients with the Zollinger-Ellison syndrome can be cured. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/3309661/Zollinger_Ellison_syndrome__Current_concepts_in_diagnosis_and_management_ L2 - https://www.nejm.org/doi/10.1056/NEJM198711053171907?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -
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