CLINICS 2012;67(S1):145-148 pancreatectomy have been proposed as surgical alternatives for the treatment of gastrinomas/MEN1 (18). However, as reported in the literature and also supported by our own observations, such surgical approaches may be frequently followed by a low cure rate and a high recurrence rate. Considering that most gastrinomas/MEN1 are located in the duodenum, a more radical surgical intervention is proposed for these patients including pancreatoduodenect- omy followed by regional lymphadenectomy (17,20). Pancreatoduodenectomy may be performed with pylorus preservation or including gastric resection (Whipple’s technique). It is important to actively search for the presence of duodenal tumors even in the first 1–2 cm of the duodenum. Despite the functional advantages of the pylorus-preserving technique, it may be safer to perform Whipple’s procedure in gastrinoma/MEN1 patients. In some cases, total pancreatectomy is the treatment of choice, in an attempt at the complete removal of all tumor lesions (Figure 2). Radical resection may also be considered because the sensitivity of the methods used in detecting tumor lesions is low. Also, intraoperative gastrin measure- ments may be helpful to improve our capacity for determining the extent of resection (14). INSULINOMAS For sporadic insulinomas the diagnosis, preoperative localization, and surgical approach are well established and surgery consists of tumor enucleation (11). In insulinoma/MEN1, the surgical management is com- pletely different because in this condition, there are multiple tumors spread throughout the pancreas, including tumors with potential for malignancy. Enucleation is not the best treatment for these lesions because high incidences of recurrence or persistence of hypoglycemia have been reported after simple tumor enucleation. The current therapeutic surgery for insulinoma/MEN1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of lesions located in the head of the pancreas (Figure 3). This procedure frequently has few postoperative complications (11,14). However, in cases with major involvement of the Figure 2 - Octroscan showing gastrinomas disseminated through- out the pancreas. Total pancreatectomy was performed in this case. Surgical treatment of pancreatic endocrine tumors in MEN1 Machado MCC 147 Figure 3 - Distal pancreatectomy in a patient with insulinomas associated with multiple endocrine neoplasia type 1. Eleven small tumors were found. Serum glucose returned to normal after surgery. pancreatic head, a pylorus-preserving pancreatoduodenect- omy should be performed. Higher recurrence rates have been observed after surgical resection of insulinoma/ MEN1, compared with sporadic insulinomas (21–23). The confirmation of complete tumor removal is of paramount importance. Intraoperative determinations of serum glucose and insulin levels for evaluating the presence of tumor tissue left behind have been reported but their accuracy is low. In our experience, intraoperative ultrasonography asso- ciated with monitoring of serum levels of glucose/insulin is a useful tool to confirm the completeness of tumor resection (11). GLUCAGONOMAS, SOMATOSTATINOMAS, AND VIPOMAS These three types of tumors occur rarely in MEN1 patients. Specific guidelines for the surgical treatment of these lesions are not currently available so the surgical procedures usually follow basically the same criteria used for non-functioning or functioning PETs described above. Most of these tumors are malignant and metastatic lesions are usually present in PETs.3 cm. Radical surgical resec- tion is the proposed treatment for these latter lesions. HEPATIC METASTASES As stated by the National Comprehensive Cancer Network guideline and the consensus guidelines by the European NET Study Group: surgical treatment is the most effective therapeutic method with which to treat hepatic metastases from PETs/MEN1 (24–26). Surgical resection of a single hepatic metastasis may be curative. Even in cases with multiple liver metastases, surgical resection is possible. In this latter situation, portal vein embolization is used as a strategy to increase the left lobe size and metastatic lesions can then be removed in a first-stage operation (Figure 4). This procedure is useful as cytoreductive surgery in patients with a clinical syndrome that is refractory to clinical treatment. Hepatic trans-arterial chemo-embolization can also be used as an ablation tool,