Zollinger–Ellison syndrome

Zollinger–Ellison syndrome

This syndrome is mentioned here because the gastrin - producing endocrine tumour is often found in the duodenal loop, although it also occurs in the pancreas, especially the head. It is a cause of persistent peptic ulceration. Before the development of potent gastric antisecretory agents, the condi - tion was recognised by sometimes fulminant peptic ulceration - that did not respond to gastric surgery short of total gastrec - tomy . The advent of PPIs such as omepraz ole has rendered this extreme endocrine condition fully controllable, but also - less easily recognised. Gastrinomas may be either sporadic or associated with the autosomal dominantly inherited multiple endocrine neopla - sia (MEN) type I (in which a parathyr oid adenoma is almost invariable). The tumours are most commonly f ound in the ‘gastrinoma triangle’, which is defined by the junction of the cystic duct and common bile duct superiorly , the junction of the second and third parts of the duodenum inferiorly and the junction of the neck and body of the pancreas medially (essentially the superior mesenteric artery). Many are found - in the duodenal loop, presumably arising in the G cells found in Brunner’s glands. It is extremely important that the duode - nal wall is carefully inspected endoscopically and at operation. ects into the medial wall of the duodenum. Even malignant sporadic gastrinomas may have a very indolent course. The palliative resection of liver metastases may be beneficial and, as for other gut endocrine tumours, liver transplantation is practised in some centres with reason ab le long-term results. However, a minority of tumours found to the left of the superior mesenteric artery (outside the ‘trian gle’) seem to have a worse prognosis, with more having liver metastases at presentation. In MEN type I, the tumours ma be multiple and the condition is incurable. Even in this situ ation, surgical treatment should be employed to remo ve any obvious tumours and associated lymphatic metastases, as good palliation may be achieved (see Chapter 57 ).