Peutz-Jeghers综合征合并肿瘤分析26例  

Peutz-Jeghers syndrome with tumors:an analysis of 26 cases

作　　者：吴绍新[1] 林军[1] 

机构地区：[1]武汉大学中南医院消化内科,湖北省武汉市430071

出　　处：《世界华人消化杂志》2008年第3期332-334,共3页World Chinese Journal of Digestology

摘　　要：目的:分析Peutz-Jeghers综合征及合并癌变的临床特征,总结其治疗和随访方案.方法:回顾性分析荆楚理工学院附属第一、二临床医院1986-09/2006-08间收治的26例Peutz-Jeghers综合征患者的临床资料.结果:10例患者(38.46%)家族史明确,临床以皮肤黏膜色素沉着、腹痛及便血为主要表现.并发肠梗阻14例(53.85%),消化道出血10例(38.46%);发生恶变6例(23.08%),其中结肠癌3例,胃癌3例,平均确诊年龄31岁,恶变者的病理组织学分型均为低分化黏液腺癌.内镜下高频电凝息肉切除术、开腹肠道息肉切除及肠部分切除术为主要治疗手段.结论:Peutz-Jeghers综合征患者是恶性肿瘤的高发人群,肿瘤患者发病年龄轻,分化较差.定期复查内镜,以内镜高频电凝积极处理肠息肉,以及筛查肿瘤是提高Peutz—Jeghers综合征远期疗效的有效方法.AIM： To analyze Peutz-Jeghers syndrome with tumors and its treatment modalities and followup program. METHODS： Retrospective clinical data on 26 cases admitted to the First and Second Affiliated Hospitals of Jingchu University of Technology from September 1986 to August 2006 were analyzed. RESULTS： Ten patients （38.46%） had a clear family history of Peutz-Jeghers syndrome. The main manifestations were skin and mucosa pigmentation, abdominal pain and hematochezia. Bowel obstruction was found in 14 patients （53.85%）, alimentary tract hemorrhage in 10 patients （38.46%）, and malignant diseases in 6 patients （23.08%） （colonic cancer in three patients and gastric cancer in the other 3 patients）. The average age of the patients at the diagnosis was 31 years. The pathohistology showed poorlydifferentiated mucinous adenocarcinomas in all the patients. The main treatment modalities used were high frequency electric polypectomy under endoscope, polyposis intestinalis polypectomy and partial excision of the bowel. CONCLUSION： Peutz-Jeghers syndrome patients have a high morbidity of cancer. PeutzJeghers syndrome occurs at a young age. Repeated endoscopy, high frequency electric polypectomy and regular screening for cancer can improve its long term curative effect.

关 键 词：PEUTZ-JEGHERS综合征 胃肠道肿瘤 合并症 症状 诊断 治疗 病例 黑斑息肉病 

分 类 号：R735[医药卫生—肿瘤]