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目的　探讨遗传性非息肉病性结直肠癌(HNPCC)的家系发病特点和预后。方法　收集11个符合Amsterdam标准的HNPCC家系,绘制其家系图谱,分析HNPCC的发病特点及预后。结果　HNPCC发病率为1%。11个家系中,共有肿瘤病人39例(多发癌5例) ,发病年龄5～79岁。其中结直肠癌灶2 9个,肠外相关肿瘤16个。在11个家系的先证者中,HNPCC的平均发病年龄为4 7岁。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5 4 .5 %。术后生存6年以上者6例,其中2例超过9年。结论　HNPCC以中分化腺癌多见,预后较好,相关肿瘤以胃癌多见。

Hereditary nonpolyposis colorectal cancer: an analysis of 11 kindreds

Objective To explore clinical features and prognosis of patients with hereditary nonpolyposis colorectal cancer (HNPCC).Methods Eleven kindreds of HNPCC according with Amsterdam standard were collected and their pedigree trees were drawn.Clinical features and prognosis of 11 kindreds of HNPCC were analyzed.Results The morbidity of HNPCC was 1%.Among 11 kindreds,there were 39 cases of cancer including 5 cases of multiple cancers.The age of patients was between 5 and 79.Among all patients,there were 29 foci of colorectal cancer and 16 foci of HNPCC related cancers.Among the propositi of 11 kindreds,the average incidence age was 47.Pathologic type of 6 propositi was tubular adenocarcinomas with well differentiation.Six cases had survived for more than 6 years including 2 cases survived for more than 9 years.Conclusion Chinese patients with HNPCC have special characteristics such as well differentiation,good prognosis and high incidence of related tumors in stomach.