遗传性非息肉病性结直肠癌7个家系的临床分析

目的:总结遗传性非息肉病性结直肠癌(HNPCC)家系的临床特点,提高临床诊断和治疗水平。方法:收集7个HNPCC家系的病例资料,分析其发病特点并记录随访结果。结果:7个HNPCC家系共有癌症患者23例,肿瘤灶27处:大肠外瘤灶3处;大肠瘤灶24处,其中有13处位于脾曲近侧结肠,占54.1%。平均发病年龄为41.2岁,17例(73.5%)发病在50岁以前。1家系累及连续三代人、4家系累及连续两代人。多原发癌患者4例,其中3例为肠外癌。结论:HNPCC具有发病年龄轻、垂直传递、肠外癌发病率高、常见多原发癌、好发于右半结肠、病理分化较差的特点,但预后相对较好。

Diagnosis and treatment of hereditary nonpolyposis colorectal cancer: an analysis of 7 families

Objective: To study the clinical characteristics of hereditary nonpolyposis colorectal cancer(HNPCC) so as to evaluate the diagnosis and treatment of this disease.Methods: Twenty three patients from 7 HNPCC families were analyzed.Results:All 23 patients and 27 malignant neoplasms were found in 7 HNPCC families,among which 3 were extracolonic cancers,while 24 were colonic cancers.Cancers located proximal to the splenic flexure accounting for 54.1%.The median age of confirmed diagnosis was 41.2 years.1 familie transmitted vertically to 3 generations,while 4 families 2 generations.There were 4 multiple colorectal cancer patients.Conclusion: HNPCC patients had the characteristics of early onsetting,high incidences of extra colorectal cancer,poor differentiation,frequent multiple colorectal cancers and the predominance of right-sided colorectal cancers.But HNPCC has a better prognosis comparing with common colorectal cancer.