Recommendation

Surgery is the treatment of choice in MEN 1 pancreatic tumors if no extensive extrapancreatic (hepatic) spread is present. Because for both MEN 1 gastrinomas and MEN 1 insulinomas the procedures are standardized, localization is of limited importance. In MEN 1 gastrinomas, the procedure of choice is probably distal pancreatectomy, exploration of the duodenum after DUODX, enucleation of tumors of the pancreatic head, and regional lymph node dissection (according to Thompson and colleagues).48 We and other authors recommend49 a pylorus-preserving pancreaticoduodenectomy because most MEN 1 gastrinomas are situated in the head of the gland (gastrinoma triangle) and duodenal gastrinomas almost always recur after local excision. Therefore, localization of tumors within the pancreas is of little value. This is more so in MEN 1 insulinomas, for which distal pancreatectomy and enucleation of tumors from the head of the gland can be called a standard procedure. Therefore, localization procedures make sense only to show tumors in the head.

On the basis of the studies mentioned previously, we recommend using preoperative US or CT and SRS to localize large tumors and to show liver metastases and other extrapancreatic metastatic spread; we also recommend ES preoperatively to localize tumors outside the pancreatic region resected.

In patients with recurrent or persistent disease, other localization techniques such as the Imamura procedure are useful.

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