Zollinger-Ellison syndrome (ZES) is a clinical syndrome with severe
peptic ulcer disease and
diarrhea caused by gastric acid hypersecretion secondary to a neuroendocrine tumour that secretes excessive amounts of the
hormone gastrin (
gastrinoma).
Gastrinomas occur in a familial and a sporadic form. Patients with
gastrinoma in the familial setting of
Multiple Endocrine Neoplasia type 1 (MEN-1) are seldom, if ever, cured of
Zollinger-Ellison syndrome by the current non-Whipple operations to remove duodenal and pancreatic
gastrinoma. Surgery is currently used in these patients to deal with the malignant nature of pancreatic or duodenal neuroendocrine tumours. Malignant potential is best determined by tumour size. Tumours that are greater than 2 cm in size should be excised. In the sporadic setting, cure occurs in a significant proportion of patients (50%) by surgical resection of
gastrinoma. Duodenotomy has improved both the tumour detection rate and the cure rate and should be routinely done. Whipple
pancreaticoduodenectomy results in the highest probability of cure in both sporadic and MEN-1
gastrinoma patients as it removes the entire
gastrinoma triangle. However, the excellent long-term survival of these patients with lesser operations and the increased operative mortality and long-term morbidity of Whipple make its current role unclear until further studies are done.