eMedicine Specialties > Gastroenterology > Stomach

Zollinger-Ellison Syndrome: Treatment & Medication

Author: Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Coauthor(s): Homayoun Shojamanesh, MD, Former Fellow, Digestive Diseases Branch, National Institutes of Health
Contributor Information and Disclosures

Updated: Jul 5, 2006

Treatment

Medical Care

  • The goals of treatment are medical control of gastric acid hypersecretion and surgical resection of the tumor.
    • If the patient is acutely ill, immediate control of gastric acid hypersecretion can be achieved with intravenous proton pump inhibitors. Previously, this was accomplished with histamine 2 (H2) receptor blockers. Intravenous pantoprazole was approved recently by the US Food and Drug Administration. Proton pump inhibitors are superior to H2 blockers for the control of gastric acid hypersecretion.
    • Patients who are candidates for surgical resection should be referred for resection of the tumor.
    • For patients with metastatic disease, chemotherapy, interferon, and octreotide may be helpful. The response to these agents in most studies has been low. Liver transplantation for hepatic metastasis also has been reported. For patients with a single confined liver metastatic lesion, surgical resection may be attempted.

Surgical Care

  • All patients with sporadic ZES without hepatic metastases or medical contraindications to surgery are advised to undergo surgical resection of the tumor because this decreases the risk of developing liver metastases, which can decrease the survival of these patients.
  • The role and timing of surgical resection in patients with MEN 1 is less clear. An attempt at surgical resection has been recommended if the tumor is larger than 2.5 cm. Cure is rarely achieved by surgical resection in patients with MEN 1; however, it may reduce the risk of subsequent metastatic disease.
  • Because this is a rare tumor, surgical resection should be attempted only at centers with personnel experienced in treating patients with ZES.

Consultations

  • Gastroenterologist
  • Surgeon
  • Oncologist
  • Possibly, endocrinologist

Medication

Medical therapy is aimed at control of gastric acid hypersecretion.

Proton pump inhibitors

Drugs of choice in ZES. Inhibit gastric acid secretion by inhibition of the H+/K+/ATP-ase enzyme system in the gastric parietal cells.


Omeprazole (Prilosec)

Decreases gastric acid secretion by inhibiting parietal cell H+/K+ ATP pump. Aim of therapy is to maintain BAO <10 mmol 1 h prior to next dose.

Adult

40 mg PO bid, titrate up prn to desired BAO

Pediatric

Not established

May decrease effects of itraconazole and ketoconazole; may increase toxicity of warfarin, digoxin, and phenytoin

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Bioavailability may increase in elderly patients


Lansoprazole (Prevacid)

Decreases gastric acid secretion by inhibiting parietal cell H+/K+ ATP pump. Aim of therapy is to maintain BAO <10 mmol 1 h prior to next dose.

Adult

60 mg PO bid, titrate up prn to desired BAO

Pediatric

Not established

May decrease effects of ketoconazole and itraconazole; may increase theophylline clearance

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

Consider adjusting dose in liver impairment


Pantoprazole (Protonix)

Decreases gastric acid secretion by inhibiting parietal cell H+/K+ ATP pump. Aim of therapy is to maintain BAO <10 mmol 1 h prior to next dose.

Adult

40-160 mg PO qd; alternatively, 80 mg IV bid

Pediatric

Not established

May decrease effects of ketoconazole and iron salts

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Decrease dose in hepatic impairment, half-life can increase 7- to 9-fold; no dose adjustment required in patients with renal impairment


Esomeprazole magnesium (Nexium)

S-isomer of omeprazole used for symptomatic GERD. Inhibits gastric acid secretion by inhibiting H+/K+ ATP pump at secretory surface of gastric parietal cells.

Adult

20-80 mg PO qd

Pediatric

Not established

Amoxicillin or clarithromycin may increase plasma levels when used concurrently; may reduce absorption of dapsone; may increase levels of diazepam and GI absorption of digoxin; may decrease absorption of iron, ketoconazole, and itraconazole

Pregnancy

C - Safety for use during pregnancy has not been established.

Precautions

Symptomatic relief with proton pump inhibitors may mask symptoms of gastric malignancy


Rabeprazole sodium (Aciphex)

Decreases gastric acid secretion by inhibiting the parietal cell H+/K+ ATP pump. For short-term (4-8 wk) treatment and relief of symptomatic erosive or ulcerative GERD. In patients not healed after 8 wk, consider additional 8-wk course.

Adult

20-80 mg tab PO qd

Pediatric

Not established

May decrease effects of itraconazole and ketoconazole; may increase toxicity of warfarin, digoxin, and phenytoin

Pregnancy

B - Usually safe but benefits must outweigh the risks.

Precautions

Symptomatic relief with proton pump inhibitors may mask symptoms of gastric malignancy

More on Zollinger-Ellison Syndrome

Overview: Zollinger-Ellison Syndrome
Differential Diagnoses & Workup: Zollinger-Ellison Syndrome
Treatment & Medication: Zollinger-Ellison Syndrome
Follow-up: Zollinger-Ellison Syndrome
References
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Further Reading

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Keywords

gastrinoma, ZES, gastrointestinal mucosal ulceration, GI mucosal ulceration, gastrin-secreting tumor, gastrin, multiple endocrine neoplasia type 1, MEN 1, malabsorption, diarrhea, heartburn, pancreas tumor, pancreatic tumor

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Contributor Information and Disclosures

Author

Praveen K Roy, MD, Comments and Criticisms Editor, Cochrane Colorectal Cancer Group
Praveen K Roy, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and Canadian Association of Gastroenterology
Disclosure: Nothing to disclose.

Coauthor(s)

Homayoun Shojamanesh, MD, Former Fellow, Digestive Diseases Branch, National Institutes of Health
Homayoun Shojamanesh, MD is a member of the following medical societies: American Gastroenterological Association, American Medical Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Medical Editor

Anil Minocha, MD, FACP, FACG, Clinical Professor, School of Pharmacy, Professor of Medicine, Director of Digestive Diseases, Medical Director of Nutrition Support, Medical Director of Gastrointestinal Endoscopy, Internal Medicine Department, University of Mississippi Medical Center
Anil Minocha, MD, FACP, FACG is a member of the following medical societies: American Academy of Clinical Toxicology, American Association for the Study of Liver Diseases, American College of Forensic Examiners, American College of Gastroenterology, American College of Physicians, American Federation for Clinical Research, American Gastroenterological Association, and American Society of Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Noel Williams, MD, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada
Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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