Patients and Methods: Seven MEN-1 patients were referred to our Surgical Unit (1992-2008) and were operated on for hypoglycemic crisis, associated with hypergastrinism in 3 patients and/or nonfunctioning pancreatic endocrine neoplasias present in 6 patients. Preoperative tumor localization was carried out using ultrasonography, computed tomography, endoscopic ultrasonography (EUS) and somatostatin receptor scintigraphy. Intraoperative ultrasonography (I0US) and intraoperative insulin measurement by rapid assay were also employed.
Results: All the pancreatic lesions larger than 0.5 cm encountered at surgery were removed by resection of the most affected pancreatic regions and by enucleation of nodules (4 pts) in least affected regions. Two Pancreatoduodenectomies and five distal pancreatectomy were performed, with no postoperative mortality. Complications ensued in 3 patients: pancreatic fistula (2pts), abdominal collection (1 pt), and acute pancreatitis (1 pt). EUS was the most sensitive preoperative imaging technique (sensitivity 71 %), while 10US proved to be the most sensitive imaging technique (86%) and intraoperative insulin assay predicted postoperative outcome in all patients. At a mean follow-up of 66 months, all patients were normoglycemic with no evidence of disease recurrence.
Conclusions: MEN-1 insulinomas should be considered surgically curable diseases. Pancreatic resection is preferable to a less radical surgical approach in ensuring higher cure rates with limited morbidity. The choice of the type of pancreatic resection should depend on the localization of the pancreatic endocrine neoplasias, on their relationship with the Wirsung duct and on the presence of other duodeno-pancreatic lesions. 10US and intraoperative insulin assay may be of value both in the assessment of the surgical decision and in the evaluation of the surgical cure for the patient.