ZES or Zollinger Ellison syndrome is a disorder which is characterized by the tumor formation in the gastrointestinal tract. Tumor generally occurs in the head of the pancreas or in the small intestine especially in the duodenum, or in both of the places. This tumor produces excess gastrin hormone which in turn stimulates the acid producing cells of the stomach to produce excess gastric acid, leading to peptic ulcers in the duodenum. The tumor is termed as Gastrinoma as it secretes Gastrin.

Gastrinomas occur as single tumors or as small, multiple tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that commonly spread to the liver and nearby lymph nodes. A number of patients with gastrinomas have many tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients often have tumors of the pituitary gland and parathyroid gland, as well as tumors of the pancreas. Gastrin works on stomach parietal cells causing them to secrete more hydrogen ions into the stomach lumen. Gastrin also acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Therefore there is an increase in the number of acid-secreting cells, and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach and duodenum. High acid levels lead to multiple ulcers in the stomach and small bowel. The disease may occur at any time in life, but the average age at diagnosis is 50 years. Disease has sexual preponderance; males are slightly more affected as compared to the women.

Signs and Symptoms

• Heartburn mimicking GERD
• Diarrhea
• Gastric discomfort
• Weakness
• Weight loss
• Blood in stools or dark colored stools
• Nausea
• Vomiting
• Jaundice
• Patients may be pale
• Epigastric tenderness
• Dental erosions may be present
• Hepatomegaly in some cases is indicative of hepatic metastases

Diagnosis and Investigations

• Blood investigations are performed to determine the levels of the hormone gastrin in the stomach. The test is performed empty stomach
• Secretion stimulation blood test is also performed in some cases, in this test injection of secretin is administered to the patient and the blood is examined for the increase in the levels of Gastrin
• Upper GI Endoscopy is performed in some cases and biopsy may be taken
• Imaging studies like CT and MRI may be of some help in some cases
• Endoscopic Ultrasound is also recommended in some cases

Treatment and Medications

• Mainstay of the treatment is group of drugs called proton pump inhibitors which effectively reduce the production of the acid from the gastric cells
• For some patients surgical resection of the tumor is performed
• Mainstay of the treatment is supportive, complications are treated symptomatically and promptly.