Reference Work Entry

Encyclopedia of Cancer

pp 1373-1373

Date:

Familial Adenomatous Polyposis

Definition

FAP is an autosomal dominant disease characterized by the presence of at least 100 polyps in the large bowel and by several extracolonic manifestations, which include polyps in other organs, desmoid tumors, dental abnormalities, osteomas, and congenital hypertrophy of the retinal pigmented epithelium. An inherited disease of the large intestine marked by the formation of numerous glandular polyps that typically become malignant if they are left untreated. In most cases it is caused by inactivation of the APC gene (APC gene in Familial Adenomatous Polyposis).

APC Gene in Familial Adenomatous Polyposis

Colorectal Premalignant Lesions

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