Summary

Gastrinomas are rare neuroendocrine tumors that elaborate excessive amounts of the hormone gastrin. Clinically, this leads to a condition known as ZES. Gastrinomas are usually sporadic, but in 20% of cases, they occur in an inherited fashion, called MEN type I. Sporadic gastrinomas tend to be solitary, with the primary tumor in the duodenum. In the setting of MEN type I, gastrinomas are also small and multiple and commonly involve the duodenum. Indeed, in both circumstances, 80% are found within the gastrinoma triangle (head of the pancreas and duodenum). Diagnosis is based on measurement of fasting serum levels of gastrin and BAO. Secretin stimulation test confirms the diagnosis. Localization of tumor relies on SRS and CT as the best initial studies. The use of intraoperative localization techniques such as IOE or duodenotomy is necessary during surgical exploration to localize small tumors within the wall of the duodenum. Currently, with the use of PPIs, there is excellent medical control of symptoms, and total gastrectomy is no longer indicated. With control of symptoms, the development of metastatic disease has become the major determinant of survival. Although slow growing, up to 60% of gastrinomas are malignant, and surgery remains the only potentially curative treatment for

Figure 14-20. Kaplan-Meier survival curves for a group of patients undergoing resection of small, single liver metastases in addition to removal of the primary gastrinoma. Resection of localized (ideally single) metastatic foci has a significant impact on survival in these patients.

these tumors. Current chemotherapy and radiation therapy regimens do not seem to prolong survival. Surgery should be considered in all patients with localized gastrinoma, although in patients with ZES and MEN type I, it is seldom curative. Even in select patients with metastatic liver disease, surgery can cure some patients and enhance survival.

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