Surgical Strategy for Insulinomas in Multiple Endocrine Neoplasia Type I

Background: Hyperinsulinemia in multiple endocrine neoplasia type I (MEN-I) is a rare but potentially curable condition that presents difficulties not encountered in sporadic cases. Methods: The present report documents our surgical approach to 3 MEN-I patients with hyperinsulinemia. Results: Primary hyperparathyroidism was manifested in all 3 patients at the time of presentation. Distal subtotal pancreatectomy with enucleation of tumor at the head of pancreas detected intraoperatively resulted in immediate cure of 2 patients. Persistent disease occurred in 1 patient after enucleation of tumor at the head of the pancreas guided by preoperative imaging elsewhere. The patient was subsequently cured by distal subtotal pancreatectomy. Pathology revealed multiple tumors (4 to 14) in all patients. Conclusions: A different surgical strategy with an aim of distal subtotal pancreatectomy and enucleation of any tumor identified in the head of pancreas is the treatment of choice for hyperinsulinemia in MEN-I patients.