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Early Age of Onset, Multiple primary Malignancies and Poor Prognosis Are Indicative of an Inherited Predisposition to Esophageal Squamous Cell carcinoma in Familial Rather Than Sporadic Disease- An Update Based on 14- to 23-year Follow-up

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Clinical oncology and cancer researeh 2009年第6期6卷 439-445页

作者： Shijie Wang Denggui Wen Liwei Zhang Lizhen Wei Wendi Zou Peng Qin Department of Endoscopy Hebei Tumor Hospital Shijiazhuang 050011 Hebei Province China Department of Epidemiology Hebei Cancer Institute Shijiazhuang 050011 Hebei Province China

OBJECTIVE To demonstrate the effects of an inherited predisposition to familial esophageal squamous cell carcinoma （ESCC） through the comparison and analysis of the clinicopathologic differences between familial and sporadic ESCC cases. METHODS Differences in age of onset, prevalence rates of double primary ESCC, and survival rates between familial ESCC （n = 476） and sporadic ESCC cases （n = 1226） were analyzed. RESULTS Overall, familial ESCC cases showed a significantly younger age of onset （51.9±8.2 vs. 53.4 ±8.0, *** = 0.00）, a significantly higher prevalence rate for double ESCC （2.73 % vs. 1.22%, adjusted with TNM：χMH2 = 4.029, P = 0.045）, and a lower survival rate than in sporadic cases （Pwald = 0.04）. The familial cases showed both a younger age of onset and poorer survival in most subgroups, and the differences were more marked in early-stage rather than in the .late-stage disease groups. CONCLUSION Theses findings confirm the existence of familial as opposed to sporadic ESCC. By the theory of the ＂two-hit＂ origin of cancer, these findings also suggest that the ＂first hit＂, a genetic predisposition, can affect the age of onset, number of primary carcinomas, and the prognosis for familial ESCC patients.

关键词： esophageal squamous cell carcinoma familial cancer age of onset synchronous primary carcinoma prognosis sporadic cancer.

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Earlier onset and multiple primaries in familial as opposed to sporadic esophageal cancer

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World Journal of Medical Genetics 2014年第2期4卷 39-45页

作者： Xiao-Duo Wen Deng-Gui Wen Yi Yang Bao-En Shan Shi-Jie Wang Department of Diagnosis and Image 4th Hospital of Hebei Medical University Cancer Center 4th Hospital of Hebei Medical University

AIM: To study the differences in onset age and multiple primary cancers between familial and sporadic esophageal squamous cell carcinoma(ESCC).METHODS: The differences in onset age and multiple primary cancers were analyzed between ESCC patients with(n = 766) and without(n = 1776) a family history of the cancer. The cases analyzed constituted all consecutive patients who had undergone cure-intent surgery at the Department of Thoracic Surgery of the 4th Hospital of Hebei Medical University from January 1 1975 to December 31 1989. Because we also originally aimed to examine the difference in survival time, only older subjects with a long follow-up period were ***: Overall, patients with ESCC and a positive family history of the cancer had a significantly younger age at onset and more multiple primary cancers than those without a positive family history(51.83 ± 8.39 vs 53.49 ± 8.23 years old, P = 0.000; 5.50% vs 1.70%, P = 0.000). Both of these differences were evident in subgroup analyses, however, no correlations were observed. While age at onset differed significantly by family history in males, smokers, and drinkers, the difference in multiple primary cancers by family history was significant in nonsmoking, nondrinking, and younger onset patients. In multivariate analysis, age over 50 years, tobacco smoking, and multiple primary cancers were found to be significant predictors of familial cancer: the corresponding OR(95%CI) and P-value were 0.974(0.963-0.985) and 0.000; 1.271(1.053-1.535) and 0.012; and 4.265(2.535-7.176) and 0.000, ***: Patients with ESCC and a positive family history of the cancer had a significantly younger onset age and more multiple primary cancers than those without a positive family history. Sub-group analyses indicated that younger onset age may be due to the interaction of genetic predisposition and environmental hazards, and multiple primary cancers may only be due to genetic predisposition.

关键词： Esophageal squamous cell carcinoma Familial cancer Sporadic cancer Age at onset synchronous primary carcinoma

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食管癌高发区贲门癌,胃癌流行强度分析(英文)

食管癌高发区贲门癌,胃癌流行强度分析(英文)

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第五届全国中医药免疫学术研讨会——暨环境·免疫与肿瘤防治综合交叉会议

作者： WEN Denggui,WANG Shijie,ZHANG Liwei,Wang Xiaolin,WEI Lizhen,ZHOU Wendi,QIN Peng Epidemiology,The Fourth Hospital of Hebei Medical University,Hebei Medical Universi Shijiazhuang

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 *** 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 *** 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 ***: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 *** difference in long-trem survival rates between the familial and the sporadic cases is analysed with the Cox propotional Hazard ***:Overall,familial ESCC cases show a significantly earlier onset age(51.9±8.2 versus 53.4±8.0 years old,P=0.00),a significantly higher prevalence rate of double ESCC(2.73%Versus 1.22%,adjusted with TNM:X=4.029,P=0.045),and a lower survival curve than the sporadic cases(P=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 ***: 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

关键词： Esophageal Squamous Cell carcinoma Familial Cancer Age of Onset synchronous primary carcinoma Prognosis Sporadic Cancer

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