NEW YORK, Aug 25 (Reuters Health) -- Surgery to remove hormone-producing tumors can cure patients with a rare gastrointestinal disorder called Zollinger-Ellison syndrome, according to a report in The New England Journal of Medicine.
The researchers recommend that surgery should be offered to all patients with the Zollinger-Ellison syndrome who do not also have a related disorder known as multiple endocrine neoplasia type 1 or metastatic disease. In these patients, a study showed that surgery did not result in cure.
Dr. Jeffrey A. Norton, of the University of California, San Francisco, and colleagues studied 151 patients with Zollinger-Ellison syndrome -- characterized by hard-to-treat peptic ulcer disease, hyperacidity in the stomach and pancreatic islet-cell tumors -- for an average of 8.4 years following their first operation for the disorder.
Hormone-producing tumors called gastrinomas were detected during surgical exploration in 93% of patients, despite the fact that imaging studies were negative in approximately one third of patients. The tumors were located in the duodenum (the first part of the small intestine) and pancreas in the majority of patients, 49% and 24%, respectively. However, 11% of patients had primary tumors in the lymph nodes and 9% had primary tumors at other sites, highlighting the importance of extensive operative exploration and whole-body imaging before surgery, the investigators explain.
"The disease-free rate immediately after resection was 51%, and it was sustained in the majority of patients during follow-up," Norton and colleagues note. Approximately one-third (34%) of patients who did not have multiple endocrine neoplasia type 1 were disease-free at 10 years. In contrast, none of the patients with multiple endocrine neoplasia type 1, a syndrome that features tumors in multiple endocrine organs, were disease-free, they add.
Overall survival for the patients in the study was 94% 10 years after surgery, the study authors report.
"Our results support the conclusion that exploratory (surgery) should be performed routinely in patients with... gastrinomas if diffuse hepatic metastases are not present," Norton and colleagues write. "Conversely, patients with multiple endocrine neoplasia type 1 rarely become free of disease, even after extensive duodenal exploration; therefore, surgical exploration for cure of the gastrinoma is not routinely recommended in these patients."
SOURCE: The New England Journal of Medicine 1999;341:635-644.
