Care of patients and their families with familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is a dominantly inherited disorder tha t is typically characterized by the appearance of multiple colorectal adeno mas usually by the teenage years, with a risk of early colorectal cancer ap proaching 100%. Genetic testing can help determine which family members hav e the disorder and require surveillance endoscopy. Astute physicians may de tect unsuspected FAP in patients with extraintestinal manifestations such a s hard or soft cutaneous tumors, Colectomy mill prevent cancer but is often necessary before the patient is 20 years old, Postoperative lifelong surve illance is indicated to screen for associated duodenal, thyroid, and rectal or ileal neoplasms. Attenuated FAP variants are less typical and mag be co nfused with other types of familial colorectal neoplasia, Chemoprevention, regression, and other treatment strategies are being developed to improve t he management of extracolonic neoplasms and desmoid tumors, A better unders tanding of the natural history of these FAP-associated phenomena will facil itate the rational selection of interventions, Management guidelines that w ere recently developed at Mayo Clinic Rochester to provide for uniform care and surveillance are discussed.