GASTRINOMAS IN MEN-1

About 33% of gastrinomas are associated with the MEN-I syndrome [17-22]. Gastrinomas in MEN-I patients present a variety of unique problems. The tumors are usually multiple, and often small and undetectable [17, 18, 20, 23, 24]. Most cases are discovered at a younger age than in sporadic cases. Although these tumors are less frequently (7-12%) malignant, the frequency of malignancy may truly be considerably higher than this. Because of the multiplicity of the tumors and their small size, it is generally quite difficult to find the specific tumor(s) that is/are secreting gastrin. Thus, the likelihood of surgical cure in MEN-I patients is considerably less than in sporadic patients, unless a specific site or sites responsible for the hypergastrinemia has been identified preoperatively.