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Verification of agermline mutation requires more intensive therapy due to more aggressive disease. Crucial for risk assessment is a detailed family history, including creation of a pedigree with cancer family history if necessary. In high-risk families, genetic counselling and annual prostate-specific antigen (PSA) screening beginning at the age of 40 should be performed. Verification of a germline mutation requires more intensive therapy due to more aggressive disease. This study evaluates surgical outcomes of Ivor Lewis esophagectomy (