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目的 探讨遗传性非息肉病性大肠癌(HNPCC)家系的临床特征。方法 对符合Amsterdam标准的4 5个HNPCC家系共2 6 4个患者绘出其家系图,确定年龄、性别、肿瘤部位、同时和异时癌等。结果 4 5个家系中2 6 4例患者,男14 2例,女12 2例,均为常染色体显性遗传;确诊时的中位年龄为5 0岁。30 5个原发癌灶中,大肠癌灶180个,大肠多发癌2 8例,肠外癌12 5个。结论 HNPCC垂直传递特征突出,肠外癌以肺癌、子宫内膜癌、胃癌最多见,同时多原发癌和异时多原发癌比较多见。
Objective To investigate the clinical features of hereditary nonpolyposis colorectal cancer (HNPCC) pedigrees. Methods A total of 246 pediatric patients with 45 HNPCC families who met the Amsterdam criteria were drawn their pedigree charts to determine the age, sex, tumor location, simultaneous and heterologous cancer. Results Among 465 pedigrees, 264 cases were male, including 142 cases and 122 cases female, all with autosomal dominant inheritance. The median age at diagnosis was 50 years. In 30 5 primary foci, there were 180 colorectal foci, 28 colorectal carcinomas and 125 extraintestinal cancers. Conclusion HNPCC is characterized by vertical transmission. Parenteral cancer is the most common form of lung cancer, endometrial cancer and gastric cancer. Multiple primary cancers and multiple primary cancers are more common.