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Zollinger-Ellison Syndrome

Douglas S. Parks, MD Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Zollinger-Ellison syndrome (ZES) triad

    • Markedly elevated gastric acid secretion

    • Peptic ulcer disease

    • A gastrinoma or non-β islet cell tumor of the pancreas or duodenal wall that produce...

DIAGNOSIS

HISTORY

Average of 5 years of symptoms (including recurrent ulcers) before diagnosis is made 
  • Abdominal pain is the most common symptom (80%).

  • Diarrhea (postprandial and fasting) (70%)

  • Heartburn...

TREATMENT

GENERAL MEASURES

  • Goals are to control acid hypersecretion and resect the tumor.

  • Advanced imaging initially to evaluate for resection

  • Surgical removal when primary tumor can be identified and as...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Longitudinal follow-up to evaluate for metastases

  • Titrate medical therapy to control symptoms.

  • Advise patients of potential danger of stopping ant...

REFERENCES

1
Corey B, Chen H. Neuroendocrine tumors of the stomach. Surg Clin North Am.  2017;97(2):333–343. [View Abstract on OvidMedline]
2
Berna MJ, Hoffmann KM, Long SH, et al. Serum gastrin in ...

ADDITIONAL READING

  • Hirschowitz BI, Fineberg N, Wilcox CM, et al. Costs and risks in the management of patients with gastric acid hypersecretion. J Clin Gastroenterol.  2010;44(1):28–33. [View Abs...

CODES

ICD10

E16.4 Increased secretion of gastrin 

ICD9

251.5 Abnormality of secretion of gastrin 

SNOMED

53132006 Zollinger-Ellison syndrome (disorder) 

CLINICAL PEARLS

  • Consider ZES if peptic ulcers recur or if high doses of PPI are needed to control symptoms/ulcers.

  • ~25–30% of cases of ZES occur in association with MEN1.

  • Once ZES is diagnosed, search f...

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