A four-decade follow-up of one of the first familial polyposis kindreds to be studied in America emphasizes the hereditary aspects and natural history of this affliction. Five of six children with familial polyposis subsequently died of colorectal cancer at ages 20, 16, 18, 15, and 23. Stringent pathologic criteria have led to newer concepts of familial polyposis; adenomatous polyps are limited to the colon and rectum, while the polypoid lesions noted in the terminal ileum are due to submucosal lymphatic hyperplasia. One or more extra-colonic lesions such as osteomas, dental abnormalities, desmoid tumors, or epidermoid inclusion cysts will be found frequently. Fibrous tissue overgrowth, especially when intramesenteric, may lead to morbidity and mortality. Duodenal and periampullary lesions appear to be the second most common malignancy in patients with familial polyposis. Colectomy with preservation of the rectum or proctocolectomy with permanent ileostomy are the current operative procedures of choice.
AMILIAL POLYPOSIS OF THE COLON IS A HE-
F redi tar): disease transmitted as an autosomal dominant and characterized by the appearance of numerous adenomatous polyps and subsequent adenocarcinoma of the large intestine at a relatively early age.86
Although there were a number of isolated case reports from America prior to 1935, Mc-Kenney's thorough family studies were among the first available in this c o ~n t r y . 1 Q ~~~ This paper constitutes a follow-up of a familial polyposis kindred first recognized by McKenney in 1930, and reported by him in 1936 and 1939. An abbreviated up-dated pedigree of this kindred was reported, in 1955, by OBrien and Wells.24 This familial polyposis kindred is interesting in several respects. First, the disease continues to manifest itself earlier than in any other documented kindred. Second, one member of this family succumbed to unrelieved colostomy stenosis resulting from an undiagnosed desmoid tumor. Third, these patients have been followed for the past four decades From the Department of Surgery, Roswell Park Memorial Institute, 666 Elm St., Buffalo, N.Y., and the