Zollinger-Ellison syndrome (ZES)

Zollinger-Ellison syndrome (ZES) is characterized by peptic ulcers, diarrhea, and marked gastric acid hypersecretion in association with a gastrin-secreting non–β islet cell endocrine tumor (gastrinoma).

-The mean age of onset of symptoms is 41 years

-Multiple endocrine neoplasia syndrome type 1 (MEN 1) is present in approximately 20–25% of patients. It consists of pancreatic neuroendocrine tumors, pituitary tumors, and hyperparathyroidism.

-Most gastrinomas occur in the submucosa of the duodenum.

-Most patients have a typical duodenal ulcer.

Symptoms and Signs: Peptic ulcer disease, abdominal pain, and diarrhea, gastroesophageal reflux, nausea, weight loss, bleeding.  

Diagnosis:

When the diagnosis is suspected, a fasting gastrin is usually the initial test performed. Serum gastrin concentration >1000 pg/mL in combination with acidic stomach pH <2.0 is diagnostic.

The secretin stimulation test is the provocative test of choice used to differentiate patients with gastrinomas from other causes of hypergastrinemia and to establish the diagnosis of ZES.

In all patients with ZES, a serum PTH, Prolactin, LH, FSH, GH levels should be obtained to exclude MEN 1.

CT and MRI scans to identify the site of the primary tumor and whether there is metastasis.

Treatment:

Medical Treatment: PPIs are the drugs of choice, though H2 blocking drugs can also be used. Nasogastric aspiration of stomach acid stops the diarrhea.

-With long-term PPI use in ZES patients, vitamin B12 deficiency can develop; thus, vitamin B12 levels should be assessed during follow-up.

-Long-term PPI use may be associated with a number of side-effects including; an increased incidence of bone fractures;

Surgical Treatment: Tumor resection

In patients with MEN 1, surgical resection is recommended

Q: What are the most common symptoms experienced by ZES patients? Abdominal pain and diarrhea

Q: What do you suspect in patients who had peptic ulcers in unusual locations? ZES

Q: Who can have significant hypergastrinemia beside individuals with ZES? Patients taking PPIs, those with H pylori infection, chronic atrophic gastritis, pernicious anemia, hypochlorhydria

Q: What is the most sensitive method for identifying ZES? Demonstration of an increased fasting serum gastrin concentration.

Q: What is the most important predictor of survival in ZES?  The presence of hepatic metastases.

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