目的　探讨遗传性非息肉病性结直肠癌 (ＨＮＰＣＣ)的临床特点和诊断。方法　收集 2 2个符合Ａｍｓｔｅｒｄａｍ标准的ＨＮＰＣＣ家族 ,分析其临床特点。结果　本组符合Ａｍｓｔｅｒｄａｍ标准的ＨＮＰＣＣ发病率为 2 .6 %,2 2个家族有恶性肿瘤患者 10 1例 ,结直肠癌患者 84例 Objective To investigate the clinical features and diagnosis of hereditary nonpolyposis colorectal cancer (HNPCC). Methods Twenty-two HNPCC families with Amsterdam criteria were collected and their clinical features were analyzed. Results The incidence of HNPCC meeting the criteria of Amsterdam was 2.6% in this group, 101 cases of malignant tumor in 22 families, 84 cases of colorectal cancer