Familial polyposis of the colon. A four-decade follow-up.

Abstract

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 extracolonic 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.