Ⅰ型多发性内分泌肿瘤综合征相关胰岛素瘤诊治分析

Analysis of diagnosis and treatment of insulinomas in multiple endocrine neoplasia Type 1(MEN-1) patients

目的探讨Ⅰ型多发性内分泌肿瘤综合征(MEN-1)相关胰岛素瘤的诊断与外科治疗策略。方法回顾性分析北京协和医院自1995年1月至2010年1月诊治有完整病历资料的合并MEN-1的胰岛素瘤21例临床资料。结果共收集到21例,男6例,女15例。6例有胰腺内分泌肿瘤或MEN-1家族史。9例为多发性,2例为恶性胰岛素瘤。其他腺体中甲状旁腺受累最为常见,其次为垂体、肾上腺。19例接受手术治疗,根据高激素水平对机体影响严重程度采用一期手术、分期手术等多种治疗方案。胰腺手术中肿瘤摘除术应用最为常见,共切除病灶58个,65.5%病灶直径<1cm。5例病人发生B级胰瘘,保守治疗后痊愈。结论 MEN-1相关胰岛素瘤有其特殊临床特点,对于高危人群应提高警惕,术中应全面探查胰腺,配合术中超声、血糖监测等方法,保证切除全部病灶,对于其他受累腺体可一期或后续处理,需与相关科室加强协作及建立必要的转诊制度。

Objectives To investigate the strategy for diagnosis and surgical procedure of insulinoma in multiple endocrine neoplasia Type 1（MEN-1） patients based on a retrospective study; Methods Medical records of patients diagnosed as MEN-1 related insulinoma from 1995 to 2010 in Peking Union Medical College Hospital were reviewed retrospectively. Clinical data was collected and statistically analyzed; Results A total of 21 patients were diagnosed as MEN-1 related insulinomas, which including 6 males and 15 females, 6 of them had family history of pancreatic endocrine tumor or MEN-1. Multiple tumors were found in 9 cases; 2 cases were malignant insulinomas. Parathyroid was the most common affected gland in these patients, followed by pituitary and adrenal gland.19 patients underwent operations, the lesions were resected in one-stage or subsequent stage based on the severity of high hormone levels harmfulness. Tumor enucleation was the most common operative procedure. In total, 58 lesions were resected, of which 65.5% are 1 cm in size. Pancreatic fistula （Grade B） occurred in 5 patients, all these patients recovered by conservative treatment; Conclusions MEN-1 related insulinomas have special clinical characteristics, much more attentions should be paid to the high risk populations. Comprehensive exploratory surgery, intraoperative ultrasonography and blood glucose testing can help to remove all lesions. Other involvement glands can be treated in one or the following stage treatment, Surgeons should collaborate with relevant departments and some difficult cases can transferred to the big-volume centers.