摘要
<正> OBJECTIVE To demonstrate the effects of an inheritedpredisposition to familial esophageal squamous cellcarcinoma (ESCC) through the comparison and analysis of theclinicopathologic differences between familial and sporadic ESCCcases.METHODS Differences in age of onset, prevalence rates ofdouble 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 significantlyyounger age of onset (51.9 ± 8.2 vs. 53.4 ± 8.0, P_(t-test) = 0.00), asignificantly higher prevalence rate for double ESCC (2.73 % vs.1.22%, adjusted with TNM:χ_(MH)~2= 4.029, P = 0.045), and a lowersurvival rate than in sporadic cases (P_(wald) = 0.04). The familial casesshowed both a younger age of onset and poorer survival in mostsubgroups, and the differences were more marked in early-stagerather than in the late-stage disease groups.CONCLUSION Theses findings confirm the existence of familialas opposed to sporadic ESCC. By the theory of the 'two-hit' originof cancer, these findings also suggest that the 'first hit', a geneticpredisposition, can affect the age of onset, number of primarycarcinomas, and the prognosis for familial ESCC patients.
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, Pt.test = 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.
基金
supported by grants from the National Scientific Support Program during the Eleventh Five-year Period (No.2006BAI02A0)
the Hebei Provincial Program for the Subjects with High Scholarship and Creative Research Potential in Ordinary Colleges and Universities
the Natural Scientific Foundation of Hebei Province (No.C2005000797)
the International Science and Technology Cooperation Item of Hebei Province (No.09396105D).
关键词
鳞状细胞癌
食管癌
早期阶段
家族性
遗传易感性
发病
恶性肿瘤
年龄
esophageal squamous cell carcinoma, familial cancer age of onset, synchronous primary carcinoma, prognosis, sporadic cancer.