Description
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.