Zollinger-Ellison Syndrome

Synonyms

Gastrinoma; ZES

Definition and Characteristics

Zollinger and Ellison described a syndrome characterized by ulcerations in the upper jejunum, hypersecretion of gastric acid, and non-beta islet cell tumors of the pancreas [1]. Gastrin produced by those tumors called gastrinoma has been identified as the humoral agent responsible for this syndrome. Today, in most of the cases, diagnosis is established before development of the complications of peptic ulcer disease or spread of a malignant gastrinoma, and therapy that modifies the course of the disease is almost always possible. Mortality largely depends on tumor dignity and the extent of disease involvement.

Prevalence

The Zollinger-Ellison syndrome (ZES) has an incidence of up to 1% in patients with a peptic ulcer disease [2]. Gastrinomas can either be sporadic or associated with so-called multiple endocrine neoplasia (MEN) type 1, an autosomal dominant predisposition to tumors of the parathyroid glands, anterior pituitary, and...