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

Study aim: The aim of this retrospective study conducted by the much less t han Groups d'etude des neoplasies endocriniennes multiples type 1 much grea ter than (GENEM) was to report a series of insulinomas associated with mult iple endocrine neoplasias type 1 (NEM 1) and to determine the most appropri ate 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 morpholo gical investigations, tumoral features, modalities and results of the treat ment. Results: Insulinomas were associated with other functional islet tumors in 15 patients: ZE syndrome (n = 8), glucagonoma (n = 6), vipoma (n = 1). Mali gnant lesions were present in 6 patients: isolated insulinomas (n = 2), ins ulinomas associated with ZE syndrome (n = 2) and vipoma (n = 1). The sensiv ity of the preoperative imaging procedures was less than 70 %. Fourty-one p atients were operated on: subtotal pancreatectomy (n = 26 including cephali c enucleations in 8), enucleations (n = 8), total pancreatectomy (n = 3), p ancreaticoduodenectomy with caudal enucleations (n = 1), and for 3 patients , no details were available. There was no postoperative mortality. Hyperins ulinism disappeared in 27 patients (including 2 after reoperation) with a m ean follow-up of 9 years. Among 6 patients with malignant lesions, 3 were a live with a 3, 4 and 10 year-followup. Conclusion: Subtotal pancreatectomy with splenic conservation and enucleati on of cephalic lesions is suggested as the procedure of choice in this grou p of patients. This procedure allows simplification of preoperative imaging investigations because lesions of the pancreatic head have only to be dete cted. Preoperative endoscopic ultrasonography and intraoperative ultrasonog raphy are the best investigations. (C) 2000 Editions scientifiques et medic ates Elsevier SAS.