1 702 例食管鳞状上皮癌家族史与发病及预后关系的研究*

目的:通过研究家族史阳性或阴性与食管鳞癌发病年龄、多原发癌灶以及预后的关系，揭示遗传易感性在食管鳞癌发生发展过程中的作用。方法:对河北医科大学第四医院1985 年1 月至 1994 年12 月手术切除的来自高发区的476 例家族史阳性和 1 226例家族史阴性食管鳞癌的发病年龄、原发癌灶数量和生存曲线进行比较。结果:全组病例家族性食管鳞癌比散发鳞癌发生年龄显著提前（51 .9±8.2 vs 53 .4±8.0，P t-test=0.00 ），双灶鳞癌发生率显著升高（2.7% vs 1.2%，adjusted with TNM:χ2MH=4.029，P=0.045）；生存时间显著降低（Pwald =0.04 ）。亚组分析多数显示家族性食管鳞癌与散发鳞癌之间发病年龄和生存曲线具有差别，发病年龄差别较大的亚组，生存率的差别较明显；发病年龄和预后的关系密切，如在Tis 、T1N0M0、T2，3N0M0 和T2-4N1M0 组发病年龄差别的t 检验、P 值分别为0.01 、0.01 和0.09 ；生存曲线的 ward 检验 P 值分别为 0.01 、0.52 和0.18 。结论:本文用临床病理和生存资料证实，高发区食管鳞癌的发生存在遗传易感性，该遗传易感性可理解为肿瘤二次突变学说中的第一次突变，对食管鳞癌的发生和预后都有影响。 

Analgsis of the Relationship between Familial History and Esophageal Squamous Cell Carcinoma in 1702 cases

Obejective: To investigate the role of familial history in the development of esophageal squamous cell carci-noma. This article analyzed the clinicopathologic differences in onset age, prevalence of double primary malignancy, and long-term post-surgical survival between familial cases and sporadic cases. Methods:There were476 cases in the familial group and 1226 cases in the sporadic group. T-test was used to explore the difference in onset age between the two groups. Chi-square test was employed to examine the prevalence of double primary ESCC. The difference in long-trem sur-vival rate between the two groups was analyzed with Cox proportional Hazard model.Results: Compared with the sporadic cases, familial ESCC cases had earlier onset age ( 51.9 ± 8.2 versus 53.4 ± 8.0 years, P t-test=0.00), higher prevalence of dou-ble ESCC (2.73% versus1.22%, adjusted with TNM: X2 MH=4.029 , P=0.045 ) and lower survival (Pwald=0.04). Conclusion:Our findings suggest the existence of an inherited genetic alteration in the familial ESCC cases. The systemic clinicopathologic differences between familial and sporadic ESCC cases revealed that the genetic predisposition can affect the onset age, the number of primary carcinoma, and the prognosis.