Pancreatic neuroendocrine tumors: gastrinoma
What is a gastrinoma/what is Zollinger-Ellison syndrome?
Gastrinoma is a type of pancreatic neuroendocrine tumor, which is a rare endocrine tumor that occurs sporadically as well as part of inherited familial endocrine syndromes such as Multiple Endocrine Neoplasia Type-1 (MEN-1). Gastrinomas occur in approximately 10 per million of the population. Gastrinoma is often associated with increased stomach acid as part of the Zollinger-Ellison Syndrome (ZES). ZES is a condition where increased production and release of stomach acid is associated with the formation of ulcers. This is an uncommon cause of stomach and duodenal ulcers (peptic ulcers) as most are actually caused by bacteria called Helicobacter pylori (H. pylori). Most peptic ulcer disease caused by H. pylori can be treated effectively with acid reducing medications and antibiotics. ZES can be distinguished from the common cause of peptic ulcer disease in most cases, namely the presence of multiple or atypical ulcers. Atypical or multiple ulcers strongly suggest the diagnosis of ZES and therefore a gastrinoma, however in 18% to 25% of patients no ulcers are present at the time of diagnosis.