非典型遗传性非息肉病性结直肠癌临床病理特征(19个家系随访)  

Thc Clinicopathological Features of l9 Nontypical Pedigress of Hereditary Nonpolyposis Colorectal Cancer.

作　　者：金黑鹰[1] 崔龙[1] 孟荣贵[1] 刘飞[1] 冯跃[1] 徐洪莲[1] 黄炜[1] 喻德洪[1] 阎于悌[2] 王金穗[2] 

机构地区：[1]第二军医大学附属长海医院肛肠外科,200433 [2]甘肃省人民医院肛肠外科,730000

出　　处：《外科理论与实践》2001年第6期393-394,共2页Journal of Surgery Concepts & Practice

基　　金：本课题为国家自然科学基金资助项目;批准号:39970823

摘　　要：目的:了解国人非典型遗传性非息肉病性大肠癌(HNPCC)的临床及病理特点,并对诊断标准进行讨论.方法:回顾和随访1980～2001年我院及协作医院诊治的非典型HNPCC病人共19家系38例;结果:第一个癌的发病平均年龄为49岁,直肠多见,占65.8%;33例进行了根治性手术,切除率为86.9%;术后病理粘液癌为5例(13.2%),Dukes分期A期2例,B期17例,C期9例,D期10例,一年、3年、5年及10年的生存率为86.02%、61.70%、38.11%及25.81%.结论:非典型HNPCC是一种发病早的大肠癌,Amsterdam标准作为HNPCC的工作标准过于严格.ve:To study the clinico-pathologic features of nontypical hereditary nonpolyposis coloretal cancer(HNPCC) in the Chinese and to discuss its diagnostic criteria. Methods: The data of 38 cases from 19 pedigreeswith nontypical HNPCC were analysed. Results: The onset of the first cancer occurred at an average age 49.Rectal cancer accounted for 65.8%, 33 cases were radically resected with a resectability of 86.9%. Dukes' sta-ging: Stages A,B,C and D comprised 2, 17, 9 and 10 cases respectively. The 1, 3, 5 and 10 year survival rates were86.02%, 61.70%, 38.11% and 25.81% respectively. Conclusious: Nontypical HNPCC is an early onset cancer.The Amsterdam criteria are too strict to be applied to HNPCC.

关 键 词：遗传性非息肉病性结直肠癌 临床病理特征 家系随访 大肠癌 HNPCC 

分 类 号：R735.3[医药卫生—肿瘤]