We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management. From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed. Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 - 68 years) were identified. Twelve patients (86%) had insulinoma, two (14%) had gastrinoma. One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization. Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected. Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail. Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV. Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy. 100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived. The overall disease free 10-year survival was found to be about 81%. Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.
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