Abstract
Familial adenomatous polyposis (FAP) is an autosomal dominant disease with a prevalence of about one in 10,000 live births. It is classically characterized by the development of hundreds to thousands of adenomas in the colon and rectum that progress to colorectal cancer (CRC) in almost all individuals if left untreated. It typically presents in early adolescence and without any intervention, 95 % of patients will develop CRC by age 50. Adequate colonoscopy surveillance and prophylactic colectomy have reduced the incidence of CRC cases in FAP. This article examines the epidemiology, clinical presentation, genetic basis, differential diagnosis, endoscopic and surgical management of FAP.
Original language | English (US) |
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Title of host publication | Intestinal Polyposis Syndromes |
Subtitle of host publication | Diagnosis and Management |
Publisher | Springer International Publishing |
Pages | 173-195 |
Number of pages | 23 |
ISBN (Electronic) | 9783319281032 |
ISBN (Print) | 9783319281018 |
DOIs | |
State | Published - Jan 1 2016 |
Externally published | Yes |
Keywords
- AFAP
- APC mutation
- FAP
- Hereditary colon cancer
- Polyposis
ASJC Scopus subject areas
- Medicine(all)