Familial Adenomatous Polyposis.
Familial adenomatous polyposis (FAP), caused by a germline mutation in the adenomatous polyposis coli (APC) gene on chromosome 5q21, is an autosomal dominant disorder characterized by hundreds to thousands of adenomas throughout the gastrointestinal tract. A variety of extraintestinal manifestations, including thyroid, soft tissue, and brain tumors, may also be present. These patients inevitably develop colorectal carcinoma by the fourth decade of life. In this review, the pathology, epidemiology, and genetic features of FAP are discussed.
Department of Pathology, Baylor University Medical Center, Dallas, Texas, United States.
Alexia
A
Waller
School of Medicine and Dentistry, Pennsylvania State University, Hershey, Pennsylvania, United States.
Sarah
S
Findeis
Department of Pathology and Cell Biology, Columbia University Medical Center, New York, New York, United States.
Michael J
MJ
Lee
Gardner syndrome; Turcot syndrome; familial adenomatous polyposis