Zollinger-Ellison Syndrome In Men1 Patients: Medical Or Surgical Treatment?

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is characterized by the occurrence of tumors in different endocrine organs mainly parathyroid glands, pancreatic islets and anterior pituitary glands. The pancreatoduodenal neuroendocrine tumors (NET) have a high penetrance with a prevalence of 9%, 53% and 84% at 20, 50 and 80 years of age, respectively [1]. Pancreato-duodenal NETs may secrete hormones that provoke a clinical syndrome of hormonal excess or not secrete hormones (non-functioning NETs). Gastrinomas are the most frequent functioning pancreato-duodenal NET which can cause gastric acid hypersecretion with the manifestation of the Zollinger-Ellison syndrome (ZES). The hypergastrinemia has a throphic effect both on gastric mucosa and on gastric enterochromaffin cells (ECL). It is diagnosed in at least 50% of MEN1 patients at an age of 50 years ca, with prevalence in men [2,3]. At the moment of the diagnosis, pancreatic non-functioning NETs are usually detectable in all patients [2]. The great majority of the MEN1-gastrinomas (>90%) are found in the deep layer of the duodenal mucosa within the Brunner’s glands or in the duodenal submucosa. This aspect is in contrast to the sporadic gastrinoma that is found prevalently in the pancreas [4]. MEN1 associated duodenal gastrinomas are usually multiple, less than 5 mm in diameter, and well differentiated with a low K1 67 (less than 2%) [5]. The metastatic potential of most duodenal gastrinomas is restricted to the peripancreatic lymph nodes which are positive in 34% to 85%. These lymph node metastases do not adversely affect survival [2,5-8] There is general agreement that duodenal gastrinomas have exceptionally fast growth or metastatisation to the liver.