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<正>Obejective:The familial aggregation of esophageal squamous cell carcinoma（ESCC） epidemiologically observed in the northern Chinese high-risk region may be argued to reflect either genetic predisposition or common household exposure.Although some molecular evidences such as single nucleartide polyphorphism is available which suggest the existence of genetic predisposition for ESCC,there is a lack of clear pathoclinical data supporting this.To demonstrate the effect of inherited predisposition for familial esophageal squamous cell carcinoma cases as opposed to the sporadic counterpart,this article analysed the clinicopathological differences in onset age,prevalence of double primary malignancy,and long-term post-surgical survival rates between familial and sporadic cases.Methods:Differences in age of onset,prevalence rates of double primary ESCC,and survival rates were analyzed between the familial（N=476） and sporadic ESCC cases（N=1226）.T-test is used to test the difference in onset ages, and Chi-square test is employed to examine the different prevalence rates of double primary ESCC.The difference in long-trem survival rates between the familial and the sporadic cases is analysed with the Cox propotional Hazard model.Results:Overall,familial ESCC cases show a significantly earlier onset age(51.9±8.2 versus 53.4±8.0 years old,Pt-test=0.00),a significantly higher prevalence rate of double ESCC(2.73%Versus 1.22%,adjusted with TNM:XMH2=4.029,P=0.045),and a lower survival curve than the sporadic cases(Pwald=0.04).The familial cases showed both earlier onset and poor survival at most subgroups,and the differences were bigger in early-stage than in late-stage groups.Conclusion: Because only an inherited genetic alteration would greatly increase the chance of double primary malignancies,our findings about a early onset,more double primary malignancies,and poor prognosis for the familial as apposed to the sporadic cases suggest the existence of such an inherited genetic alteration in the familial ESCC cases.By the theory of"two-hit"origin of cancer,the systemic pathoclinical differences found between the familial and the sporadic cases also suggest that the"first hit"-the genetic predisposition,can affects the onset age,the number of primary carcinoma,and the prognosis.