Zollinger-Ellison syndrome. Spontaneous regression of advanced intra-abdominal metastases with 20 year survival

A 35-year-old man with the Zollinger-Ellison syndrome who is alive and well 20 years following diagnosis at age 15, is represented. At the initial operation for a bleeding duodenal ulcer a ZE tumor of the pancreas also was excised. After two additional ineffective ulcer operations, total gastrectomy was performed. At that time, retroperitoneal and peripancreatic metastases were noted, and several were removed. Three years later at laparotomy, extensive hepatic metastases were biopsied as well. Spontaneous remission occurred and when re-explored at age 34, after 14 years, no metastases were identifiable in any area. The serum gastrin level has remained elevated, casting doubt upon its value as a criterion for evaluation of tumor recurrence or activity. Other additional interesting aspects are discussed. The importance of elimination of the acid-secreting cells by total gastrectomy is emphasized. The patient remains well.