Zollinger-Ellison Syndrome Treatment & Management

  • Author: Praveen K Roy, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 3, 2010
 

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.
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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.
  • In a single-institution retrospective study with a median follow-up of 18 years from a diagnosis of ZES, Mortellaro et al examined the long-term outcomes in 12 patients with MEN 1 and ZES from 1970 to the present.[1] The pancreas (n = 10), duodenum (n = 4), lymph nodes (n = 3), and liver (n = 1) were the most commonly identified gastrinoma sites. A total of 15 celiotomies were performed, and surgeries included 4 each of distal pancreatectomies and acid-reducing procedures, 3 each of enucleation of pancreatic gastrinoma and duodenal resection, 1 pancreaticoduodenectomy, 7 noted as other.[1] There was 1 each of a patient with transient (3 y) biochemical postsurgical cure and liver metastasis of gastrinoma (but no deaths from metastatic gastrinoma).[1] Deaths included causes such as respiratory arrest (n = 1), possibly due to aspiration or pulmonary embolus, and nondisease related (n = 3). At last follow-up, 7 patients were alive. The investigators observed patients with MEN 1 and ZES rarely achieve biochemical cures with surgery; however, extended surgical resection was not only not needed in resection of localized gastrinomas, but it was also associated with excellent long-term outcomes.[1]
  • Because this is a rare tumor, surgical resection should be attempted only at centers with personnel experienced in treating patients with ZES.
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Consultations

  • Gastroenterologist
  • Surgeon
  • Oncologist
  • Possibly, endocrinologist
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Contributor Information and Disclosures
Author

Praveen K Roy, MD  Comments and Criticisms Editor, Cochrane Colorectal Cancer Group; Adjunct Associate Research Scientist, Lovelace Respiratory Research Institute, Albuquerque

Praveen K Roy, MD, is a member of the following medical societies: American College of Gastroenterology and American Gastroenterological Association

Disclosure: Nothing to disclose.

Coauthor(s)

Abhishek Choudhary, MD  Resident, Department of Internal Medicine, University Hospital of Missouri

Abhishek Choudhary, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Mohamed Othman, MD  Staff Physician, Department of Internal Medicine, University of New Mexico School of Medicine

Disclosure: Nothing to disclose.

Homayoun Shojamanesh, MD  Former Fellow, Digestive Diseases Branch, National Institutes of Health

Homayoun Shojamanesh, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Jack Bragg, DO  Associate Professor, Department of Clinical Medicine, University of Missouri School of Medicine

Jack Bragg, DO is a member of the following medical societies: American College of Osteopathic Internists and American Osteopathic Association

Disclosure: Nothing to disclose.

Gautam Dehadrai, MD  Department Chair, Section Chief, Department of Interventional Radiology, Norman Regional Hospital

Gautam Dehadrai, MD is a member of the following medical societies: American College of Radiology, Medical Council of India, and Radiological Society of North America

Disclosure: Nothing to disclose.

Showkat Bashir, MD  Assistant Professor, Department of Medicine, Division of Gastroenterology, George Washington University, Washington, DC

Showkat Bashir, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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.

Alex J Mechaber, MD, FACP  Associate Dean for Undergraduate Medical Education, Associate Professor of 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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