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).
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).
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DOI：
10.1016/s0001-4001(00)00112-4
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发表时间：
2000-02-01
期刊：
影响因子：
--
通讯作者：
Calender, A
中科院分区：
文献类型：
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作者：
Cougard, P;Goudet, P;Calender, A
研究目的：本回顾性研究的目的是报告一系列与多发性内分泌肿瘤1型（NEM 1）相关的胰岛素瘤，并确定最合适的局部探查和手术治疗策略。患者和方法：从1960年至1996年，纳入44例患者，16例男性和28例女性（平均年龄：36.4 ± 13.9岁）。本研究涉及形态学调查，肿瘤的特点，方式和treatment.Results的结果：胰岛素瘤与其他功能性胰岛肿瘤15例：ZE综合征（n = 8），胰高血糖素瘤（n = 6），vipoma（n = 1）。6例患者存在恶性病变：孤立性胰岛素瘤（n = 2），与ZE综合征相关的胰岛素瘤（n = 2）和血管瘤（n = 1）。术前影像学检查的敏感性低于70%。41例患者接受了手术：胰腺次全切除术（n = 26，包括8例头部眼球摘除术）、眼球摘除术（n = 8）、全胰腺切除术（n = 3）、胰管切除术伴尾部眼球摘除术（n = 1），3例患者的详细信息不详。无术后死亡。平均随访9年，27例患者（包括2例再次手术后）的高胰岛素血症消失。结论：保留脾脏的胰次全切除加头端病灶剜出术是治疗胰腺癌的首选术式。该程序可以简化术前影像学检查，因为只需检测胰头病变。术前超声内镜检查和术中超声检查是最好的检查方法。(C)2000版科学与医学Elsevier SAS。
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.