Insulinomas associated with multiple endocrine neoplasia type 1. Report of a series of 44 cases by the groupe d'etudes des neoplasies endocriniennes multiples type 1 (GENEM).

Study aim: The aim of this retrospective study conducted by the much less than Groups d'etude des neoplasies endocriniennes multiples type 1 much greater than (GENEM) was to report a series of insulinomas associated with multiple endocrine neoplasias type 1 (NEM 1) and to determine the most appropriate strategy for the topographical exploration and surgical management. Patients and methods: From 1960 to 1996, 44 patients were included, 16 men and 28 women (mean age: 36,4 +/- 13,9 years). This study concerned morphological investigations, tumoral features, modalities and results of the treatment. Results: Insulinomas were associated with other functional islet tumors in 15 patients: ZE syndrome (n = 8), glucagonoma (n = 6), vipoma (n = 1). Malignant lesions were present in 6 patients: isolated insulinomas (n = 2), insulinomas associated with ZE syndrome (n = 2) and vipoma (n = 1). The sensivity of the preoperative imaging procedures was less than 70 %. Fourty-one patients were operated on: subtotal pancreatectomy (n = 26 including cephalic enucleations in 8), enucleations (n = 8), total pancreatectomy (n = 3), pancreaticoduodenectomy with caudal enucleations (n = 1), and for 3 patients, no details were available. There was no postoperative mortality. Hyperinsulinism disappeared in 27 patients (including 2 after reoperation) with a mean follow-up of 9 years. Among 6 patients with malignant lesions, 3 were alive with a 3, 4 and 10 year-followup. Conclusion: Subtotal pancreatectomy with splenic conservation and enucleation of cephalic lesions is suggested as the procedure of choice in this group of patients. This procedure allows simplification of preoperative imaging investigations because lesions of the pancreatic head have only to be detected. Preoperative endoscopic ultrasonography and intraoperative ultrasonography are the best investigations. (C) 2000 Editions scientifiques et medicates Elsevier SAS.