History

A high index of clinical awareness is needed to make a diagnosis of Zollinger-Ellison syndrome (ZES), as its signs/symptoms are generally nonspecific.^{[1, 2]}

Abdominal pain is the most common symptom, present in 75% of patients. Typically, it is located in the upper abdomen and mimics that of peptic ulcer disease. This symptom is reported more frequently by men and patients with the sporadic form of ZES.

Of patients with ZES, 73% have diarrhea; this is the most common symptom in patients who have multiple endocrine neoplasia-type 1 and ZES (MEN 1/ZES) as well as in female patients. The combination of diarrhea and abdominal pain is present in more than half the patients.

Heartburn is the third most common symptom, and this symptom mimics gastroesophageal reflux disease (GERD).

Other symptoms include nausea, vomiting, gastrointestinal bleeding, and weight loss. Gastrointestinal bleeding frequently is due to ulceration in the duodenum and is the presenting symptom in 25% of patients.

In patients in whom MEN 1/ZES is suspected, a history indicative of nephrolithiasis, hypercalcemia, and pituitary disorders should be sought. A family history of nephrolithiasis, hyperparathyroidism, and gastrinoma also may be present.

Physical Examination

The findings of the physical examination may be normal. Note the following:

Patients may be pale if they present with gastrointestinal bleeding.
Jaundice may occur if the tumor compresses the common bile duct, although this presentation is very rare.
Epigastric tenderness may be present.
Dental erosions may be noted if symptoms consistent with gastroesophageal reflux disease (GERD) are present.
The presence of hepatomegaly suggests liver metastasis.

Differential Diagnoses

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Author

Praveen K Roy, MD, MSc Clinical Assistant Professor of Medicine, University of New Mexico School of Medicine

Praveen K Roy, MD, MSc is a member of the following medical societies: Alaska State Medical Association, American Gastroenterological Association

Disclosure: Nothing to disclose.

Coauthor(s)

Sarah D Komanapalli, MBBS Resident Physician in Internal Medicine, Marshfield Clinic

Sarah D Komanapalli, MBBS is a member of the following medical societies: American College of Physicians

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, American Society for Gastrointestinal Endoscopy

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, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Chief Editor

Philip O Katz, MD, FACP, FACG Chairman, Division of Gastroenterology, Albert Einstein Medical Center; Clinical Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Philip O Katz, MD, FACP, FACG is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Medtronic<br/>Received income in an amount equal to or greater than $250 from: Torax medical: pfizer consumer, .

Additional Contributors

Anil Minocha, MD, FACP, FACG, AGAF, CPNSS 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; Clinical Professor, University of Mississippi School of Pharmacy

Anil Minocha, MD, FACP, FACG, AGAF, CPNSS is a member of the following medical societies: American Academy of Clinical Toxicology, American Society for Gastrointestinal Endoscopy, American Federation for Clinical Research, American Association for the Study of Liver Diseases, American College of Forensic Examiners Institute, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association

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Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Jehad Barakat, MD, to the development and writing of this article.

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