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目的 探讨遗传性非息肉病结直肠癌(hereditary nonpolyposis colerectal cancer,HNPCC)的特有诊断、治疗、随访及临床特征.方法 对3个有HNPCC特征的家族进行分析.结果3个HNPCC家族中有9例大肠癌及2例合并子宫内膜癌,共有大肠癌15处(其中5例为多原发大肠癌),中位发病年龄为38岁.结论HN-PCC具有发病年龄轻、近侧结肠癌多见、同时或异时大肠癌比例高及易发生其他器官癌的特点.对HNPCC患者及其亲属进行密切随访和定期检测具有早期诊断的重要意义.
Objective To investigate the unique diagnosis, treatment, follow-up and clinical features of hereditary nonpolyposis colpectal cancer (HNPCC). Methods Three families with HNPCC characteristics were analyzed. Results There were 9 cases among the 3 HNPCC families. Colorectal cancer and 2 cases of endometrial cancer, a total of 15 colorectal cancers (5 cases of multiple primary colorectal cancer), the median age of onset was 38 years old. Conclusion HN-PCC has a younger age of onset, more proximal colon cancer The high proportion of colorectal cancer at the time of observation, simultaneous or differentiating, and the characteristics of other organ cancers. The close follow-up and regular detection of HNPCC patients and their relatives have important significance for early diagnosis.