中国人遗传性非息肉病性结直肠癌临床病理特征分析

Analysis of the clinicopathological features of Chinese hereditary nonpolyposis colorectal cancer

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 investigate the clinicopathological features of Chinese hereditary nonpolyposis colorectal cancer (HNPCC). Methods Patients who met the Amsterdam criteria were enrolled in this study from several hospitals in China. Clinicopathological features of patients with HNPCC were compared between the patients with suspected HNPCC and sporadic colorectal cancer. Results One hundred and sixty- seven individuals from 31 families met the Amsterdam criteria. The average age was 48.6( 22～ 78) years old. There were 43 cases(31.9% ) with ascending colon cancer and 52 cases(38.5% ) with rectal cancer. The 3- ,5- ,10- years survival rate was 70.3% ,49.9% and 39.7% respectively. The incidence of multiple primary neoplasms was 20.4% . Conclusions Chinese HNPCC is characterized by early disease onset. Rectal cancer and ascending colon cancer are the first and the secondly common cancer for Chinese HNPCC. Gastric cancer is the most common parenteral cancer in Chinese HNPCC families.