Zollinger–Ellison Syndrome (ZES) is caused by a non–beta islet cell (islet of Langerhans), gastrin-secreting tumor of the pancreas that stimulates the acid-secreting cells of the stomach (parietal cells) to maximal activity, with consequent gastrointestinal mucosal ulceration. ZES may occur sporadically or as part of an autosomal dominant familial syndrome called multiple endocrine neoplasia type 1 (MEN 1). The primary tumor is usually located in the pancreas, duodenum or abdominal lymph nodes, but ectopic locations have also been described (e.g., heart, ovary, gallbladder, liver, kidney).
Signs and symptoms
Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.
- Chronic diarrhea, including steatorrhea (fatty stools)
- Pain in the esophagus, especially between and after meals at night
- Vomiting blood (digested blood)
- Loss of weight due to loss of appetite
Proton pump inhibitors (omeprazole, and lansoprazole) and histamine H2-receptor antagonists (such as famotidine and ranitidine) are used to slow down acid secretion. PPI and H2 antagonist are relatively ineffective for pharmacologic treatment. Octreotide is the best drug for pharmacologic management. Cure is only possible if the tumors are surgically removed, or treated with chemotherapy.