Current Management of Zollinger-Ellison Syndrome

Summary

Patients with Zollinger-Ellison syndrome require that management decisions be made to control the gastric acid hypersecretion and treatment directed at the gastrinoma itself. The advent of newer antisecretory drugs and increased knowledge of the natural history of this disease have led to major changes in the management of each of these two areas. Recent studies have demonstrated that treatment with the currently available histamine H₂-receptor antagonists (cimetidine, ranitidine) with or without an anticholinergic agent will control gastric acid secretion in almost all patients. These studies have also shown that most patients require higher doses than those used routinely to treat peptic ulcer, treatment is only successful if an adequate dose of antisecretory drug is used and must be monitored by measuring gastric acid hypersecretion, and established criteria to regulate the dose must be used. Newer more potent antisecretory drugs such as famotidine or omeprazole will facilitate management of gastric hypersecretion but are not yet currently available.

Highly selective vagotomy should be considered in those patients who require high doses of Cimetidine or ranitidine. Total gastrectomy should be reserved for those patients unwilling or unable to take oral medication. Although aggressive surgery is not warranted in most patients because overall prognosis is excellent, tumour status should be assessed in all patients by imaging studies (CT scan, ultrasound, selective angiogram). Patients without metastatic disease and without the MEN-1 syndrome (multiple-endocrine-neoplasia type 1) should undergo exploratory laparotomy by a surgeon experienced in treating this disease, with studies suggesting a cure rate of approximately 20%. Recent studies demonstrate that patients with MEN-1 can only very rarely be cured by non-radical surgery and routine surgical exploration is not recommended. In patients with metastatic gastrinoma, at what point chemotherapy should be started has not been established.