Familial adenomatous polyposis is an important hereditary risk factor for colon cancer. Such patients and families need special attention for prevention, early detection, and optimal treatment. Molecular testing is key to identify the specific mutation in the proband and can then make it easier to identify other family members at risk. Aggressive surveillance and colonoscopy will be indicated in most patients. Both colonic and extra-colonic manifestations are important. Chemoprevention is worth considering. Almost all patients will ultimately need colectomy. These details will be discussed in this review.
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