遗传性非息肉病性结直肠癌家系临床病理特征分析  

Analysis of clinicopathological featurs in patients with Chinese hereditary nonpolyposis colorectal kindreds

作　　者：颜士岩[1] 周晓燕[1] 蔡三军[2] 于宝华[1] 罗定存[3] 杜祥[1] 施达仁[1] 

机构地区：[1]复旦大学附属肿瘤医院病理科,上海200032 [2]复旦大学附属肿瘤医院腹部外科,上海200032 [3]温州市第二人民医院肿瘤外科

出　　处：《中华消化杂志》2007年第12期813-816,共4页Chinese Journal of Digestion

基　　金：上海市医学重点学科资助项目(05Ⅲ004)

摘　　要：目的探讨中国人遗传性非息肉病性结直肠癌(HNPCC)家系发病特点及预后。方法收集24个符合Amsterdam标准的HNPCC家系,绘制其家系图谱、收集临床病理及随访资料,分析中国人HNPCC的发病特点及预后。结果24个HNPCC家系中,共有肿瘤患者116例(其中先证者多原发癌16例,家系成员多原发癌9例),发病年龄19～74岁,其中结直肠癌灶120个,肠外相关肿瘤32个。在24个HNPCC家系的先证者中,患第一结直肠癌的平均年龄为42.5岁,男性多于女性,右半结肠肿瘤多于左半结肠;肿瘤分化均较好,以中分化多见,病理类型以管状腺癌多见,占45.8%(11/24);截至随访结束时,术后生存≥5年者共14例,占58.3%(14/24),其中9例超过10年,最长1例存活时间已达27年。结论中国人HNPCC以中分化管状腺癌多见,发病年龄较轻,右半结肠癌多见。Objective To explore clinical features and prognosis of patients with hereditary nonpolyposis colorectal cancer（HNPCC）. Methods Twenty-four kindreds of Chinese HNPCC according to Amsterdam standard were enrolled and their pedigree trees were drawn. Clinicopathological and followup data were collected, clinical features and prognosis of 24 kindreds of Chinese HNPCC were analyzed as well. Results Among 24 HNPCCkindreds, there were 116 cases of cancer including 16 cases of multiple cancers in probands and 9 cases of multiple cancers in the members of kindreds. The age of patients ranged from 19 to 74. Of all the patients, there were 120 loci of colorectal cancers and 32 loci of HNPCC related extracolonic cancers. Among probands of 24 HNPCC kindreds, the average incidence age of onsetting first colorectal cancer was 42.5, the male and right-side colon cancer patiens were more than female and left-side colon cancer patiens, respectively. The most pathologic type was tubular adenocarcinomas with moderately differentiation, which accounted for 45.8 %（11/24）. Up to the deadline of follow-up, 14 cases had survived for more than 5 years accounting for 58.3 % （14/24） Of them, 9 cases survived for more than 10 years and 1 case survived for 27 years. Conclusions Chinese patients with HNPCC have special characteristics such as moderately differentiated tubular adenocarcinomas, early onset of colorectal cancer, right-side colon involvement.

关 键 词：结直肠肿瘤 遗传性非息肉病性 病理 临床 

分 类 号：R735.3[医药卫生—肿瘤]