Report of a family with hereditary site-specific colon cancer

A large family with a high prevalence of colorectal cancer, presenting the major features of hereditary site-specific colon cancer (i.e., autosomic dominant model of heredity, absence of associated adenomatous polyposis, prevalence of right-sided colonic lesions, and young age of the patients) is described. The lack of association with solitary adenomatous polyps, the higher survival rate of patients with left-sided colon cancer, and the absence of extracolonic tumors were peculiar characteristics of this family. These data, in agreement with other reports, appear to suggest that the inherited colon cancer syndromes are not a single entity, but a group of related entities linked by their familial occurrence.