Disease Information for Zollinger-Ellison syndrome/gastrinoma: Definition

  • A triad comprising intractable, sometimes fulminating, and in many ways, atypical ulcers; extremely elevated gastric acidity; and gastrin-secreting non-beta islet cell tumors, usually of the pancreas, but sometimes in other sites (eg, the duodenum), which may be single or multiple, large or small, benign or malignant (Dorland, 27th ed)------------------------------------------------------------

    Zollinger Ellison Syndrome; Gastrinoma; Pancreatic Ulcerogenic Tumor Syndrome; Z-E Syndrome; ZES

    Zollinger-Ellison syndrome (ZES) is characterized by the development of a tumor (gastrinoma) or tumors that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach; Many affected individuals develop multiple gastrinomas, approximately half to two-thirds of which may be cancerous (malignant); In most cases, the tumors arise within the pancreas and/or the upper region of the small intestine (duodenum); Due to excessive acid production (gastric acid hypersecretion), individuals with ZES may develop peptic ulcers of the stomach, the duodenum, and/or other regions of the digestive tract; Symptoms and findings associated with ZES may include mild to severe abdominal pain; diarrhea; increased amounts of fat in the stools (steatorrhea); and/or other abnormalities; In most affected individuals, ZES appears to develop randomly (sporadically) for unknown reasons; In approximately 25 percent of cases, ZES occurs in association with a genetic syndrome known as multiple endocrine neoplasia type 1 (MEN-1)----[nord website 2006]---------------------