Familial adenomatous polyposis natural history, complications and prognosis: Difference between revisions
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==Natural history== | ==Natural history== | ||
* Familial adenomatous polyposis is a rare disease that usually starts during teenage years.<ref name="KennedyPotter2014">{{cite journal|last1=Kennedy|first1=Raelene D.|last2=Potter|first2=D. Dean|last3=Moir|first3=Christopher R.|last4=El-Youssef|first4=Mounif|title=The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes|journal=Journal of Pediatric Surgery|volume=49|issue=1|year=2014|pages=82–86|issn=00223468|doi=10.1016/j.jpedsurg.2013.09.033}}</ref> | * Familial adenomatous polyposis is a rare disease that usually starts during teenage years.<ref name="KennedyPotter2014">{{cite journal|last1=Kennedy|first1=Raelene D.|last2=Potter|first2=D. Dean|last3=Moir|first3=Christopher R.|last4=El-Youssef|first4=Mounif|title=The natural history of familial adenomatous polyposis syndrome: A 24year review of a single center experience in screening, diagnosis, and outcomes|journal=Journal of Pediatric Surgery|volume=49|issue=1|year=2014|pages=82–86|issn=00223468|doi=10.1016/j.jpedsurg.2013.09.033}}</ref> | ||
* Patients with familial adenomatous polyposis develop hundreds to thousands colon polyps | * Patients with familial adenomatous polyposis usually develop hundreds to thousands [[colon polyps]] till early twenties. | ||
* They might have upper gastrointestinal | * 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 [[Polyp|polyps]] in different [[Organ (anatomy)|organs]] including [[Gastrointestinal tract|upper gastrointestinal tract]]. However, their progression to [[Cancer|malignancy]] is less than [[colon polyps]]. | |||
==Complications== | ==Complications== | ||
Complications that can develop as a result of familial adenomatous polyposis are:<ref name="IaquintoFornasarig2008">{{cite journal|last1=Iaquinto|first1=Gaetano|last2=Fornasarig|first2=Mara|last3=Quaia|first3=Michele|last4=Giardullo|first4=Nicola|last5=D'Onofrio|first5=Vittorio|last6=Iaquinto|first6=Salvatore|last7=Di Bella|first7=Simone|last8=Cannizzaro|first8=Renato|title=Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis|journal=Gastrointestinal Endoscopy|volume=67|issue=1|year=2008|pages=61–67|issn=00165107|doi=10.1016/j.gie.2007.07.048}}</ref><ref name="KingDozois2000">{{cite journal|last1=King|first1=John E.|last2=Dozois|first2=Roger R.|last3=Lindor|first3=Noralane M.|last4=Ahlquist|first4=David A.|title=Care of Patients and Their Families With Familial Adenomatous Polyposis|journal=Mayo Clinic Proceedings|volume=75|issue=1|year=2000|pages=57–67|issn=00256196|doi=10.4065/75.1.57}}</ref> | Complications that can develop as a result of familial adenomatous polyposis are:<ref name="IaquintoFornasarig2008">{{cite journal|last1=Iaquinto|first1=Gaetano|last2=Fornasarig|first2=Mara|last3=Quaia|first3=Michele|last4=Giardullo|first4=Nicola|last5=D'Onofrio|first5=Vittorio|last6=Iaquinto|first6=Salvatore|last7=Di Bella|first7=Simone|last8=Cannizzaro|first8=Renato|title=Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis|journal=Gastrointestinal Endoscopy|volume=67|issue=1|year=2008|pages=61–67|issn=00165107|doi=10.1016/j.gie.2007.07.048}}</ref><ref name="KingDozois2000">{{cite journal|last1=King|first1=John E.|last2=Dozois|first2=Roger R.|last3=Lindor|first3=Noralane M.|last4=Ahlquist|first4=David A.|title=Care of Patients and Their Families With Familial Adenomatous Polyposis|journal=Mayo Clinic Proceedings|volume=75|issue=1|year=2000|pages=57–67|issn=00256196|doi=10.4065/75.1.57}}</ref> | ||
*[[Colon cancer]] which has two different pathways: | *[[Colon cancer]] which has two different pathways according to defect on [[APC (gene)|''APC'' gene]]: | ||
**Molecular pathogenesis of sporadic colon cancer | **Molecular pathogenesis of sporadic [[Colorectal cancer|colon cancer]] | ||
[[image:Sporadic Colon Cancer3.jpg|left]] | [[image:Sporadic Colon Cancer3.jpg|left]] | ||
<br style="clear:left" /> | <br style="clear:left" /> | ||
:*Molecular pathogenesis of colitis-associated colon cancer | :*Molecular pathogenesis of colitis-associated [[Colorectal cancer|colon cancer]] | ||
[[image:Colitis-associated colon cancer.jpg|left]] | [[image:Colitis-associated colon cancer.jpg|left]] | ||
<br style="clear:left" /> | <br style="clear:left" /> | ||
*Duodenal and ampullary cancer | *Duodenal and ampullary cancer: | ||
**They are major causes of death after [[polypectomy]] | **They are major causes of death after [[polypectomy]]. | ||
**End-viewing | **End-viewing and side-viewing duodenoscopy are conducted for surveillance. | ||
==Prognosis== | ==Prognosis== | ||
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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
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.
- 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:[2][3]
- 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 after 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.
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.
- ↑ 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.