Abstract
Gastrinomas causing Zollinger-Ellison syndrome (ZES) are the most common functional, malignant pancreatic endocrine tumors. In this paper, the diagnosis and treatment of these tumors are reviewed, incorporating recent advances in each area. Furthermore, recent advances in their pathology, molecular pathogenesis, and aspects of their localization using somatostatin receptor scintigraphy, as well as their treatment are discussed. Recent data from our NIH prospective studies on patients with ZES are included to illustrate many of these points.