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Familial and sporadic esophageal cancer have different etiology, treatment, and prognosis. To discriminate between them is important to research, prevention, and treatment. This large surgical cohort presents rich data to understand familial esophageal cancer in a high-risk area in China where the cancer ranks as the No.1 or No.2 most common cancer. To concentrate studying on familial esophageal cancer cases may help to make a breakthrough in finding molecular mechanisim of inherited predisposition for esophageal cancer.