FRONTIERS IN BIOSCIENCE;
FAMILIAL POLYPOSIS



DISTINCTIVE FEATURES

An autosomal dominant disease. Variants of the disease include Gardner's syndrome (polyposis, multiple osteomas, epidermal cysts, desmoid tumors, increased risk of thyroid and duodenal carcinomas, and fibromatosis), and Turcot's syndrome (polyposis and gliomas).
Polyps start to appear in the second to third decades of life.
The risk of developing adenocarcinoma is 100%.
Prophylatic colectomy is the method of choice for preventing development of adencarcinoma of colon.
Grossly, there are numerous adenomatous polyps in the colon.
Microscopically, the adenomatous polyps show the same morphology as any other adenomatous polyp. Each polyp has a fibroconnective core and is lined by glands and a surface epithelium.