gastrointestinal disorders
Familial Adenomatous Polyposis
Key Points
Familial adenomatous polyposis is an autosomal dominant disease in which≥100 adenomatous polyps carpet the colon and rectum. Nearly all patients develop colon carcinoma by age 40, so total proctocolectomy is usually done soon after diagnosis. Patients have an increased risk of other cancers, particularly of the duodenum, and also the pancreas, thyroid, brain, and liver. After treatment, patients are screened regularly for other cancers and development of polyps in the upper gastrointestinal tract. Children of parents with familial adenomatous polyposis may be screened for hepatoblastoma from birth to age 5 years. 