Zollinger-Ellison syndrome in children: a 25-year follow-up

The influence of gastric resection on the Zollinger-Ellison syndrome (ZES) in eight children was first reported to the Central Surgical Association 26 years ago. That report showed that the three children who had less than total gastrectomy were all dead with complications of gastric acid hypersecretion, although the five children who had total gastrectomy were living and well. During the past quarter of the century, the development of effective gastric acid inhibitors has prompted a greater emphasis on medical management in the ZES. The role of the surgeon and total gastrectomy remains controversial. Follow-up of the five young patients who had total gastrectomy shows that only one patient was dead with tumor 14 years after total gastrectomy and that the remaining four patients were alive 30, 29, 28, and 27 years, respectively, after total gastrectomy. Growth and activity have been near normal. All patients have had proven metastatic islet-cell carcinoma documented at some time in the course of the disease (lymph nodes in three patients and liver metastasis in two patients). Only one of the four living patients with total gastrectomy had a normal serum gastrin level and no apparent tumor. Gastrinoma tumor growth appears to be less aggressive in children than in adults. Complete excision of gastrinomas is possible in some patients with ZES. When hypergastrinemia persists, total gastrectomy may be preferable to lifelong medical management with gastric acid inhibitors in children and young adults with ZES.