Zollinger-Ellison Syndrome Clinical Presentation

Updated: Jan 04, 2019
  • Author: Praveen K Roy, MD, MSc; Chief Editor: Philip O Katz, MD, FACP, FACG  more...
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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.