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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 ,发生第一个结直肠癌的平均年龄为 45 .7岁 ,位于脾曲近侧结肠和直肠的分别占 5 8.3%和 2 3.8%。 2 3.8%患者发生同时或异时多原发结直肠癌。 2 0例患者发生肠外肿瘤 ,以胃癌居多。结论 HNPCC具有发病年龄早、近侧结肠多见、同时和异时多原发结直肠癌发生率高的特点 ,诊断治疗及随访应有别于散发性结直肠癌。本组肠外肿瘤以胃癌发生率高 ,与国外报道不同。建立中国人的HNPCC诊断标准是必要的。
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%. There were 101 cases of malignant tumor in 22 families and 84 cases of colorectal cancer. The average age of the first colorectal cancer was 45.7 years , Located in the proximal colon and rectum of splenic flexion accounted for 53.3% and 23.8% respectively. 2 3.8% of patients with multiple primary colorectal cancer occur simultaneously or heterosexual. Twenty patients developed parenteral tumors, mostly gastric cancer. Conclusions HNPCC has the characteristics of early onset, proximal colon, and high incidence of multiple primary colorectal cancer at the same time. Diagnosis, treatment and follow-up should be distinguished from sporadic colorectal cancer. This group of extra-parenteral tumors with a high incidence of gastric cancer, and foreign reports are different. The establishment of Chinese HNPCC diagnostic criteria is necessary.