Abstract

SYSTEMATIC REVIEW OF ZOLLINGER-ELLISON SYNDROME

Jojeena Ann Mammen*, Arya Gigi and Bincy Varghese

ABSTRACT

Zollinger-Ellison syndrome (ZES) or Strom-Zollinger-Ellison syndrome is a rare disorder which is triad of gastric acid hyper secretion, severe peptic ulceration, and non-beta cell islet tumour of pancreas (gastrinoma). The syndrome is often caused due to the tumour of the duodenum or pancreas producing increased levels of hormone gastrin which produces excess hydrochloric acid in the stomach leading to ulceration in almost 95% of patients. Gastrinomas may occur as single tumours or as multiple, small tumours. About onehalf to two-thirds of single gastrinomas are malignant tumours that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumours as part of a condition called multiple endocrine neoplasia type I (MEN I) where the tumours are present in pituitary gland and parathyroid glands in addition to pancreas. The diagnosis of ZES has improved with development in biochemistry detection and advances in radiological imaging, angiography, Somatostatin receptor scintigraphy etc. The treatment of patients with Zollinger-Ellison syndrome (ZES) has undergone dramatic evolution during the past decade. Till 1970s, the only effective therapy for controlling acid hyper secretion was total gastrectomy. Currently, after the introduction of potent antisecretory drugs, such as H2 antagonists and proton pump inhibitors, chemotherapy, chemoembolisation etc has brought greater relief in the morbidity and complications related to ZES. In this article we have given an overview about the epidemiology, pathogenesis, clinical manifestations, advancement in diagnosis and management of ZES.

Keywords: Zollinger-Ellison syndrome, gastrin, gastrinomas, Multiple Endocrine Neoplasia Type.