遗传性非息肉病性大肠癌和普通遗传性大肠癌临床表型分析

The Analysis of Clinical Phenotype of the Hereditary Nonpolyposis Colorectal Cancer and the Common Hereditary Colorectal Cancer

  • 摘要: 目的:探讨遗传性非息肉病性大肠癌(HNPCC)与普通遗传性大肠癌临床表型的异同,为临床辨认HNPCC家系提供依据。方法:选择符合阿姆斯特丹标准Ⅱ或日本标准的22个HNPCC家系(A组)和普通遗传性大肠癌20个家系(B组)为研究对象进行随访分析。结果:1)A、B两组男女发病的比例分别为1.4:1(41/30)和1.5:1(38/26),无显著性差异(P>0.05)。2)A、B两组确诊的中位年龄分别为48岁(32~70岁)和61岁(30~83岁),50岁以前发病比例分别为59.2%和26.6%,A组较B组发病年龄明显提前,差异显著(P<0.01)。3)A、B两组右半结肠癌发病比例分别为56.9%(29/51)和29.2%(7/24),差异显著(P<0.05)。4)A组多发癌7例,B组未见多发癌。5)A组第一、二、三代的平均发病年龄分别为64岁、56岁、48岁,逐渐年轻化,B组无此现象。结论:HNPCC与普通遗传性大肠癌临床表型不尽相同,提示两者各自有其独特的遗传学特征。

     

    Abstract: Objective: To study the different clinical phenotypes between HNPCC and common hereditary colorectal cancer and to provide evidences to recognize the genealogical hereditary cancer of colon. Methods: A total of 22 parentages of HNPCC (A group) which is in line with the Amsterdam standard II or Japanese standard and 20 parentages of common colorectal cancer (B group) were followed- up and analyzed. Results: 1) The ratio of incidence between male and female patients of group A and B was 1.37:1 (41/30) and 1.46:1 (38/26) respectively, with no statistically significant difference(P>0.05). 2) The median age in group A and B was 48 (32~70) and 61 (30~83) years, showing a significant difference between the two groups (P<0.001). The morbidity before 50 years in the groups was 59.2% (group A) and 26.6% (group B), showing an earlier incidence in group A than in group B. 3) The ratio of right hemicolonic cancer in group A and B was 56.9% (29/51) and 29.2% (7/24), with a significant difference (P<0.05). 4) There were 7 cases of multiple cancer in group A, but none in group B. 5)The mean age of onset of the patients in the first, second, and third generation in group A was 64, 56,and 48 respectively, with a tendency of piecemeal juvenescence, but it didn' t occurred in group B.Conclusion: The clinical phenotypes of HNPCC and common hereditary colorectal cancer have many differences, which indicate some different characteristics of heritage between them.

     

/

返回文章
返回