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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。
Objective To investigate the pedigree characteristics and prognosis of hereditary nonpolyposis colorectal cancer (HNPCC). Methods A total of 11 HNPCC pedigrees meeting the Amsterdam criteria were collected and their pedigrees were drawn to analyze the characteristics and prognosis of HNPCC. Results The incidence of HNPCC was 1%. Of the 11 pedigrees, there were 39 cancer patients (5 with multiple cancers), with a mean age of onset of 5 to 79 years. There were 29 colorectal carcinomas and 16 parenteral tumors. Among 11 pedigree probands, the mean age at onset of HNPCC was 47 years. Tumor differentiation are better, more common in differentiation. Pathological type tubular adenocarcinoma more common, accounting for 54.5%. 6 patients survived more than 6 years, of which 2 cases more than 9 years. Conclusions HNPCC is more common in moderately differentiated adenocarcinoma and has a better prognosis. The related tumors are more common in gastric cancer.