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Zollinger-Ellison Syndrome

Douglas S. Parks, MD Reviewed 06/2020
 


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. Reoperation may be considered (4).

  • Titrate medical therapy to control symptoms.

  • Advise patients...

REFERENCES

1
Metz DC, Cadiot G, Poitras P, et al. Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty Gastrin assays, sensitive imaging and limited access to acid secretory testing. Int ...

ADDITIONAL READING

  • De Angelis C, Cortegoso Valdivia P, Venezia L, et al. Diagnosis and management of Zollinger-Ellison syndrome in 2018. Minerva Endocrinol.  2018;43(2):212–220.

  • Smallfield GB, All...

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 Zollinger-Ellison syndrome if peptic ulcers recur or if unusually high doses of PPI are needed to control symptoms.

  • Abdominal pain and diarrhea should be totally controllable w...

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