With the increasing recognition of the duodenum as the most common site for gastrinomas, and with the improved localization methods, at least 50% of patients with sporadic gastrinoma can be cured by tumor resection [1, 11, 13, 52]. Thus, an aggressive approach to tumor localization is useful in selecting patients for operative treatment.
The goals of treatment of gastrinoma include: 1) management of the gastric acid hypersecretion; 2) resection of what are commonly malignant tumors with the risk of distant spread, and ultimately death of the patient.
Treatment has undergone significant changes since the syndrome was originally described in 1955.[13] Most patients underwent emergency surgery for complications such as massive hemorrhage or perforation. It became rather clear that partial gastrectomy with or without vagotomy was ineffective treatment. Total gastrectomy became the standard operation for patients with gastrinoma [1, 11, 12, 17]. Although this operation was effective in controlling acid hypersecretion, many patients went on to suffer morbidity and mortality from the tumor itself. Approximately 60% of patients with gastrinoma have malignant tumors, which, although relatively slowly growing, are the major contributing factor to mortality with long-term follow-up [1]. Occasional cases in which the primary tumor was excised, usually combined with total gastrectomy, resulted in long-term cures.
The development of potent antisecretory drugs has changed the management of patients with gastrinoma [53-56]. In most patients gastric acid hypersecretion can be controlled with long-term omeprazole. Patients on long-term omeprazole can develop gastric carcinoid tumors. In addition, significant decreases in serum vitamin B12 have been observed [57]. Thus vitamin B12 levels should be monitored in these patients. Because current antisecretory agents are so effective, surgery for the control of gastric acid hypersecretion is no longer required. A study of 212 patients from the National Institute of Health at a mean follow-up of nearly 14 years showed that none of the patients suffered an acid related death.[58] Overall, 31% of patients died, one half due to the gastrinoma itself. Thus, the current role of surgery is to identify and remove the responsible tumor or tumors, to prevent tumor progression and ultimately death. In early surgical series, the cure rate was relatively modest, ranging from 15-30% [12, 20, 34, 59-62]. Those with extra-pancreatic tumors were noted to have a cure rate as high as 50% with surgical excision [1, 11, 12, 52]. With improvements in preoperative imaging studies, the increasing recognition of the duodenum as a site for gastrinoma, and an aggressive surgical approach, the number of positive explorations has increased in one group from 64% to over 90%, primarily through the identification and resection of duodenal wall gastrinomas [5, 6]. Even after successful resection, many patients do recur with long-term follow-up. In a study of 151 patients by Norton and colleagues, in patients with sporadic gastrinoma the 10 year disease free survival was 34%, and the disease specific survival was 95% [63].