The author of this letter provides further support for our contention that the familial syndrome of diverse cancers involves an autosomal dominant mode of inheritance and that the higher-than-expected percentage of affected relatives is the result of ascertainment bias. The proband and family members on the line of ascent represent only one of several potential biases in retrospective studies to assess the familial aggregation of disease. Our prospective study dealt with additional selection factors, as enumerated in the "Comments" section.Cancer develops during the lifetime of one in four persons in the United States. On a chance basis, some families show a much higher percentage of relatives with cancer while others have a low proportion affected. A selective study of the former type of families may suggest a multiple cancer syndrome when chance aggregation is the true explanation. Segregation analysis can also be misleading when study families are
Li FP, Fraumeni JF. Family Cancer Syndrome-Reply. JAMA. 1982;248(16):1972. doi:10.1001/jama.1982.03330160024009
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