Description

• Zollinger-Ellison syndrome (ZES) describes a chronic clinical syndrome of acid peptic disease due to gastric acid hypersecretion caused by a gastrinoma (a neuroendocrine tumor-secreting ectopic gastrin).
• It is often associated with severe and refractory peptic ulcers and gastroesophageal reflux disease (GERD); diarrhea, abdominal discomfort, or other symptoms may be present.
• Gastrinomas, which are typically found in the duodenum or pancreas, are most often sporadic but are associated with multiple endocrine neoplasia type 1 (MEN1) in a significant minority of patients.
• System(s) affected: endocrine/metabolic, gastrointestinal
• Synonym(s): Z-E syndrome; pancreatic ulcerogenic tumor syndrome; multiple endocrine neoplasia, partial; ulcerogenic islet cell tumor

Epidemiology

Etiology and Pathophysiology

• Gastrinomas (neuroendocrine tumors-secreting ectopic gastrin) prompt the gastric acid hypersecretion that causes ZES. Gastrinomas arise in the duodenum in 70–80% of cases, and the next most common site is the pancreas; other sites do occur rarely (1).
• Gastrinomas are neuroendocrine tumors that secrete ectopic gastrin.
• Hypergastrinemia leads to increased parietal cell mass/gastric mucosal hypertrophy and acid hypersecretion.

Risk Factors

• MEN1
• Family history of ulcer disease

General Prevention

Screen first-degree relatives of patients with MEN1.

Commonly Associated Conditions

• Gastrinoma
• MEN1 (associated with tumors of the pancreas, pituitary, and parathyroid)
• Peptic ulcer disease (PUD)
• GERD