Prognosis

Metastases

Metastatic gastrinoma is the most common cause of mortality in patients with Zollinger-Ellison syndrome (ZES). A study of patients with multiple endocrine neoplasia type 1 (MEN1) found that 31% to 57% of deaths were due to malignant gastrinoma; liver, bone, and other distant metastases were all significantly associated with MEN1-related death.[83] In a study of 185 patients with ZES who were followed prospectively for a mean of 12.5 years, the following were observed:[84]

  • At the time of diagnosis, 22% of patients without MEN1 had liver metastases; by comparison, only 6% of patients with MEN1 had liver metastases at initial diagnosis. It is unclear why this difference occurred. One possibility is that patients with known MEN1 may be diagnosed at an earlier stage in their disease.

  • Liver metastasis was significantly more frequent in patients with primary pancreatic neoplasms than in patients with duodenal tumours.

  • Patients with liver metastases had a 10-year survival of only 30%.

Without metastases

In one study, patients with sporadic disease without metastases have a 15-year survival of 85%.[84]

In patients with MEN1 without liver metastasis, the survival rate was 100% at 20 years.[84]

Another prospective study of patients with MEN1 and ZES found an overall 30-year survival rate of 82%, but noted the mean age at death was 55 years. No deaths were related to acute complications of uncontrolled gastric acid hypersecretion.[83]

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