eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Zollinger-Ellison Syndrome: Follow-up

Author: Stefano Guandalini, MD, Director, University of Chicago Celiac Disease Program, Section Chief of Gastroenterology, Hepatology and Nutrition; Professor, Department of Pediatrics, University of Chicago Comer Children's Hospital
Coauthor(s): Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine
Contributor Information and Disclosures

Updated: Dec 1, 2008

Follow-up

Further Inpatient Care

  • Even after surgery and assumed complete gastrinoma resection, patients with Zollinger-Ellison syndrome (ZES) should be kept safe on acid inhibitory medication until a secretin test has been performed and the basal acid output (BAO) has been determined.
  • Biochemical study results that are within the reference range, including serum gastrin levels less than 115 pg/mL and a secretin test with a gastrin rise less than 200 pg/mL, do not exclude a prolonged elevation of the BAO. As much as 6 months may pass before BAO decreases to reference range levels because of hypertrophy of the parietal cell mass acquired during the active phase of the disease.

Further Outpatient Care

  • Postoperative normogastrinemia does not mean long-term cure. Almost 50% of patients with postoperative eugastrinemia experience recurrence of the disease within 5 years.
  • Progression of the disease should be monitored with somatostatin receptor scintigraphy (SRS) on a yearly basis.

Inpatient & Outpatient Medications

  • Long-term proton pump inhibitor (PPI) use should be continued as indicated based on BAO.
  • In cases with advanced metastatic disease, chemotherapy with a combination of streptozocin, 5-fluorouracil, and doxorubicin may be used. A response rate of 65% can be expected.

Complications

  • With the introduction of PPIs that are effective in almost all patients, acid-related complications are now reduced to a minimum.
  • Complications of peptic ulcer disease (PUD) prior to the diagnosis include perforation, bleeding, and esophagitis. A stricture of the gastroesophageal junction may develop.

Prognosis

  • Approximately 60% of gastrinomas are benign at the time of diagnosis, without metastases. Because of the small size of gastrinomas, cure (disease free for at least 5 y) is achieved in only 20-40% of cases.
  • The development of liver metastases is considered an unfavorable sign; however, depending on the tumor biology, patients with liver metastases have survived 10 years and longer.

Patient Education

  • Families of patients who have Zollinger-Ellison syndrome with multiple endocrine neoplasia type 1 (MEN-1) should be informed about the disease and should be screened for MEN-1.

Miscellaneous

Medicolegal Pitfalls

  • Exclude multiple endocrine neoplasia type 1 (MEN-1) with tumors of the parathyroids, pancreatic islets, and pituitary. If hyperparathyroidism is present, the hypercalcemia, which is present in 95% of MEN-1 cases with gastrinomas, must be corrected with parathyroidectomy before abdominal surgery is considered.
  • Screening family members for MEN-1 is advisable.
  • Continue proton pump inhibitors (PPIs) after surgical resections until reliable measurements of basal acid output (BAO) are performed. Acid hypersecretion may persist after curative surgery for 6-12 months.

Special Concerns

  • Almost 50% of patients who are cured after surgery experience recurrence of the disease within 5 years.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Stacy Kahn, MD, to the development and writing of this article.



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
Multimedia: Zollinger-Ellison Syndrome
References
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Further Reading

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Keywords

Zollinger-Ellison syndrome, gastrinoma, ZES, multiple endocrine neoplasia type 1, MEN-1, peptic ulcers, gastrin-producing tumors, gastrinomas, neuroendocrine tumor, vasoactive intestinal polypeptide tumors, VIPomas, glucagonomas, peptic ulcer disease, PUD, abdominal pain, gastroesophageal reflux, stenosis, Barrett mucosa, proton pump inhibitor, PPI

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Contributor Information and Disclosures

Author

Stefano Guandalini, MD, Director, University of Chicago Celiac Disease Program, Section Chief of Gastroenterology, Hepatology and Nutrition; Professor, Department of Pediatrics, University of Chicago Comer Children's Hospital
Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Coauthor(s)

Klaus Radebold, MD, PhD, Research Associate, Department of Surgery, Yale University School of Medicine
Klaus Radebold, MD, PhD is a member of the following medical societies: American Gastroenterological Association and New York Academy of Sciences
Disclosure: Nothing to disclose.

Medical Editor

Jayant Deodhar, MD, Associate Professor in Pediatrics, BJ Medical College, India; Honorary Consultant, Departments of Pediatrics and Neonatology, King Edward Memorial Hospital, India
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

David A Piccoli, MD, Chief, Division of Gastroenterology and Nutrition, Department of Pediatrics, The Children's Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine
David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, State University of New York, Downstate Medical Center College of Medicine; Distinguished Lecturer, New York Medical College, School of Public Health
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
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