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目的研究中国人遗传性非息肉病性结直肠癌(HNPCC)的临床病理特征,为制定符合中国人特点的HNPCC诊断和治疗方案提供依据。方法联合全国多家医院,对符合Amsterdam标准的HNPCC进行随访,同时与疑似HNPCC患者和散发性结直肠癌患者进行对照。结果31个符合Amsterdam标准的HNPCC家系167例患者,发病年龄平均48.6(22～78)岁,升结肠癌与直肠癌多见,3、5、10年生存率分别为70.3%、49.9%和39.7%,同时性和异时性多原发癌的发生率为20.4%。结论中国人HNPCC发病早,有一定生存优势,除升结肠外,直肠癌多见,中国人肿瘤肠外谱中胃癌为最常见肿瘤。
Objective To study the clinicopathological features of hereditary nonpolyposis colorectal cancer (HNPCC) in China and provide the basis for making the diagnosis and treatment of HNPCC in Chinese. METHODS: HNPCC in accordance with the Amsterdam standard was followed up in a number of hospitals across the country, and compared with patients with suspected HNPCC and sporadic colorectal cancer. Results A total of 31 HNPCC pedigrees matched with Amsterdam standard had an average age of onset of 48.6 (22-78) years. Ascending colon and rectal cancers were more common, and their 3, 5 and 10-year survival rates were 70.3%, 49.9% and 39.7%, respectively %, Concurrent and heterochronic multiple primary cancer was 20.4%. Conclusions The incidence of HNPCC in Chinese people is earlier than others, and there is a certain survival advantage. Except for ascending colon, colorectal cancer is more common, and gastric cancer is the most common type of cancer in Chinese patients.