Familial adenomatous polyposis natural history, complications and prognosis
Familial adenomatous polyposis Microchapters |
Differentiating Familial adenomatous polyposis from other Diseases |
---|
Diagnosis |
Treatment |
Case Studies |
Familial adenomatous polyposis natural history, complications and prognosis On the Web |
American Roentgen Ray Society Images of Familial adenomatous polyposis natural history, complications and prognosis |
FDA on Familial adenomatous polyposis natural history, complications and prognosis |
CDC on Familial adenomatous polyposis natural history, complications and prognosis |
Familial adenomatous polyposis natural history, complications and prognosis in the news |
Blogs on Familial adenomatous polyposis natural history, complications and prognosis |
Directions to Hospitals Treating Familial adenomatous polyposis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sadaf Sharfaei M.D.[2], Mohamad Alkateb, MBBCh [3]
Overview
Familial adenomatous polyposis is a rare disease that usually starts from childhood. Patients develop hundreds to thousands colon polyps till early twenties. If they left untreated, almost all of them develop colorectal cancer around 40 years of age. They might have polyps in different organs including upper gastrointestinal tract. However, their progression to malignancy is less than colon polyps. They might develop duodenal and ampullary cancer which are major causes of death following polypectomy. The prognosis of familial adenomatous polyposis is excellent with treatment.
Natural history
- Familial adenomatous polyposis is a rare disease that usually starts during teenage years.[1]
- Patients with familial adenomatous polyposis usually develop hundreds to thousands colon polyps till early twenties.[2]
- They might be asymptomatic till third decade of their lives or whenever they have malignant transformation to colorectal cancer.
- If they left untreated, almost all of them develop colorectal cancer around 40 years of age.
- They might have polyps in different organs including upper gastrointestinal tract. However, their progression to malignancy is less than colon polyps.
Complications
Complications that can develop as a result of familial adenomatous polyposis are:[3][4]
- Colon cancer which has two different pathways according to defect on APC gene:
- Molecular pathogenesis of sporadic colon cancer
- Molecular pathogenesis of colitis-associated colon cancer
- Duodenal and ampullary cancer:
- They are major causes of death following polypectomy.
- End-viewing and side-viewing duodenoscopy are conducted for surveillance.
Prognosis
- The prognosis of familial adenomatous polyposis is excellent with treatment.
- Without treatment, all patients with familial adenomatous polyposis develop colon cancer at age of 40-50.[2]
References
- ↑ Kennedy, Raelene D.; Potter, D. Dean; Moir, Christopher R.; El-Youssef, Mounif (2014). "The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes". Journal of Pediatric Surgery. 49 (1): 82–86. doi:10.1016/j.jpedsurg.2013.09.033. ISSN 0022-3468.
- ↑ 2.0 2.1 Beech D, Pontius A, Muni N, Long WP (2001). "Familial adenomatous polyposis: a case report and review of the literature". J Natl Med Assoc. 93 (6): 208–13. PMC 2594024. PMID 11446392.
- ↑ Iaquinto, Gaetano; Fornasarig, Mara; Quaia, Michele; Giardullo, Nicola; D'Onofrio, Vittorio; Iaquinto, Salvatore; Di Bella, Simone; Cannizzaro, Renato (2008). "Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis". Gastrointestinal Endoscopy. 67 (1): 61–67. doi:10.1016/j.gie.2007.07.048. ISSN 0016-5107.
- ↑ King, John E.; Dozois, Roger R.; Lindor, Noralane M.; Ahlquist, David A. (2000). "Care of Patients and Their Families With Familial Adenomatous Polyposis". Mayo Clinic Proceedings. 75 (1): 57–67. doi:10.4065/75.1.57. ISSN 0025-6196.