原发性肝细胞肝癌的家族聚集性研究——附8个家系34例患者的临床分析和家系调查

The clinical analysis and family exploration of 34 cases of primary hepatocellular carcinomas.

目的总结原发性肝细胞肝癌（primary hepatocellular carcinomas, PHC）的家族聚集现象和家族性PHC的临床特点。方法回顾性分析了34例家族性PHC的临床表现，并进行了家系调查。结果34例PHC患者分布在8个家系中，平均4．25例（3．9例）；患者发病年龄平均41．3岁（32-62岁），肝炎病毒感染率94．1％（32／34），肝炎或肝硬化率61.8％（21／34）．AFP阳性率82．4％（28／34），就诊时TNM分期Ⅱ期2例，Ⅲ期23例，Ⅳ期9例；生存时间1-25月，平均7．3月。结论通过家族史明确高危人群、有重点地定期检查，提高治疗效果，为目前切实可行的PHC防治策略和思路。

Objective： To sum up clinical manifestation and familial genetics of primary hepatocellular carcinomas （PHC）. Methods ： Retrospective analysed 34 patients with PHC and explorited thire familial genetics. Results ： All 34 cases of PHC were in 8 families and every family was 4. 25 cases （3-9 eases）. The averagy years of PHC patients were 41.3 （32 -62） years old. Thief hepatitis virus rates were 94. 1% （32/34） and hepatitis/eirrosis were 61.8% （21/34）. Thief AFP positive rates were 82.4% （28/ 34）. TNM stage Ⅱ, Ⅲ,Ⅳ of PHC patients were 2, 23, 9 cases respectively. Allover survival time of them was 7. 3 monthes （ 1 - 25）. Conclusion： These results suggest that familial genetics might play an important role in earlier prevention measures and diagnosis as well as more effective therapies of PHCs.