VIPoma and PPoma

Abstract

Gastrinomas are rare tumors of the neuroendocrine system, occurring within the pancreas and duodenum. The annual incidence is estimated at 0.5 per million of the population [1]. Overproduction of gastrin by these tumors produces a sustained increase in gastric acid secretion, leading to clinical manifestations of complicated peptic ulcer disease known as the Zollinger-Ellison syndrome (ZES) [2]. Although most gastrinomas grow slowly, over 60 % are malignant and 25 % show rapid aggressive behavior [3-5]. Most commonly gastrinomas metastasize to regional lymph nodes and the liver, and it is this malignant potential which has become increasingly important since the introduction of effective medical therapy to control gastric acid secretion. Gastrinomas can occur sporadically or in a familial pattern as a component of the multiple endocrine neoplasia type 1 (MEN1) syndrome [3, 6, 7]. Given that the endocrinopathy associated with these tumors can be well controlled medically, the role of surgical resection in the setting of advanced disease and in patients with MEN1 syndrome is the subject of continued debate.