武汉地区八个家系遗传性非息肉病性结直肠癌的临床特点

Clinical Features of Hereditary Non-polyposis Colorectal Cancer in Wuhan:an Analysis of 8 Pedigrees

目的：探讨武汉地区遗传性非息肉病性结直肠癌（hereditary nonpolyposis colorectal cancer,HNPCC）家系发病情况及临床特点.方法：收集8个遗传性HNPCC家系,其中符合Amsterdam标准Ⅱ2个,Japan标准5个,Bethesda指导原则1～3项1个,分析其临床特点.结果：8个家系107人,有癌症患者20例.平均年龄53.35岁,发病高峰年龄50～59岁.20例中肠癌15例,其中右半结肠癌5例,左半结直肠癌10例;肠外癌5例.结论：武汉地区HNPCC家系与散发性结直肠癌（sporadic colorectal cancer,SCRC）相比具有发病年龄轻、肠外癌发病率高、常见多原发癌及好发于右半结肠的特点,某些特点与国内其他地区不完全相同.

Objective ：To investigate the clinical character of hereditary nonpolyposis colorectal cancer（ HNPCC ） families collected from population in Wuhan. Methods： 8 families of hereditary nonpolyposis colorectal cancer（ HNPCC ） was included in the study. Patients in the 2 families fulfilled Amsterdam Criteria（ AC ）, patients in 5 families fulfilled Japan Criteria（ JC ） and in 1 families fulfilled Bethesda Guidelines（BG） 1 - 3items. The clinical character of HNPCC were studied. Results： Among the 20 cancer patients,there were 15 colorectal cancer patients and 5 extracolorectal cancer patients. The median age in HNPCC cancer group were 53.35 years old,and the peak age in HNPCC cancer group were 50 to 59 years old. Conclusion：Compared with sporadic colorectal cancer, HNPCC patients in Wuhan has the character of earlier onsetting, high incidence of extracolorectal cancer, frequent multiple colorectal cancers and the predominance of right sided colorectal cancers, there are some differences of Hereditary non-Polyposis Colorectal Cancer between Wuhan and other areas.