Management of hereditary site-specific colon cancer

A family with site-specific colon cancer and discrete adenomatous colon polyps in certain members has provided a prototype for the study and clinical management of hereditary cancer. Analysis of this kindred showed an autosomal dominant mode of inheritance, early onset of cancer with predilection for the right colon, and frequent extraprimary cancers of the colon. Knowledge of these phenomena are critical to the management of this hereditary cancer syndrome. On our recommendation, two brothers of the family who were treated for colon cancer by hemicolectomy underwent prophylactic total colectomy. The resected colon from one of these patients had an occult adenocarcinoma in a villoglandular polyp.