Familial adenomatous polyposis natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
Familial adenomatous polyposis is a rare disease that usually starts from childhood. Patients with familial adenomatous polyposis develop numerous [[colon polyps]], which may vary from hundreds to thousands, by early twenties. If patients with FAP left untreated, almost all of the [[colon polyps]] develop [[colorectal cancer]] around 40 years of age. Patients with FAP may have [[Polyp|polyps]] in different [[Organ (anatomy)|organs]] including the [[Gastrointestinal tract|upper gastrointestinal tract]]. However, the progression of these extra colonic polyps, to [[Cancer|malignancy]] is less than [[colon polyps]]. Patients with FAP 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== | ==Natural history== | ||
Following are a few important facts about the natural history of familial adenomatous polyposis: | |||
* 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 | * Patients with FAP usually develop hundreds to thousands [[colon polyps]] by the age of early twenties.<ref name="pmid11446392">{{cite journal |vauthors=Beech D, Pontius A, Muni N, Long WP |title=Familial adenomatous polyposis: a case report and review of the literature |journal=J Natl Med Assoc |volume=93 |issue=6 |pages=208–13 |year=2001 |pmid=11446392 |pmc=2594024 |doi= |url=}}</ref> | ||
* | * Patients with FAP might be asymptomatic till third decade of their lives or whenever they have [[malignant]] transformation to [[colorectal cancer]]. | ||
* If left untreated, almost all of the patients with FAP develop [[colorectal cancer]] around 40 years of age. | |||
* Patients with FAP 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 | Complications that can develop as a result of familial adenomatous polyposis include:<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 | **1- Molecular pathogenesis of sporadic [[Colorectal cancer|colon cancer]] is as follows: | ||
[[image:Sporadic Colon Cancer3.jpg|left]] | [[image:Sporadic Colon Cancer3.jpg|left|frame|Molecular pathogenesis of colon cancer<ref name="Kim2014">{{cite journal|last1=Kim|first1=Eun Ran|title=Colorectal cancer in inflammatory bowel disease: The risk, pathogenesis, prevention and diagnosis|journal=World Journal of Gastroenterology|volume=20|issue=29|year=2014|pages=9872|issn=1007-9327|doi=10.3748/wjg.v20.i29.9872}}</ref>]] | ||
<br style="clear:left" /> | <br style="clear:left" /> | ||
:*Molecular pathogenesis of colitis-associated colon cancer | :*2- Molecular pathogenesis of colitis-associated [[Colorectal cancer|colon cancer]] is as follows: | ||
[[image:Colitis-associated colon cancer.jpg|left]] | [[image:Colitis-associated colon cancer.jpg|left|frame|Molecular pathogenesis of colitis-associated colon cancer<ref name="Kim2014">{{cite journal|last1=Kim|first1=Eun Ran|title=Colorectal cancer in inflammatory bowel disease: The risk, pathogenesis, prevention and diagnosis|journal=World Journal of Gastroenterology|volume=20|issue=29|year=2014|pages=9872|issn=1007-9327|doi=10.3748/wjg.v20.i29.9872}}</ref>]] | ||
<br style="clear:left" /> | <br style="clear:left" /> | ||
*Duodenal and ampullary cancer | *Duodenal and ampullary cancer: | ||
**They are major causes of death | **They are major causes of death following [[polypectomy]]. | ||
**End-viewing | **End-viewing and side-viewing duodenoscopy are conducted for surveillance. | ||
==Prognosis== | ==Prognosis== | ||
* The prognosis of familial adenomatous polyposis is excellent with treatment. | * The prognosis of familial adenomatous polyposis is excellent with treatment. | ||
*Without treatment, all patients with familial adenomatous polyposis develop [[Colorectal cancer|colon cancer]] at age of 40-50.<ref name="pmid11446392">{{cite journal |vauthors=Beech D, Pontius A, Muni N, Long WP |title=Familial adenomatous polyposis: a case report and review of the literature |journal=J Natl Med Assoc |volume=93 |issue=6 |pages=208–13 |year=2001 |pmid=11446392 |pmc=2594024 |doi= |url=}}</ref> | |||
*Without treatment, all patients with familial adenomatous polyposis develop [[Colorectal cancer|colon cancer]] at age of 40-50. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
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[[Category:Medicine]] | |||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
[[Category: | [[Category:Oncology]] | ||
[[Category: | [[Category:Up-To-Date]] | ||
Latest revision as of 21:43, 29 July 2020
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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
Familial adenomatous polyposis is a rare disease that usually starts from childhood. Patients with familial adenomatous polyposis develop numerous colon polyps, which may vary from hundreds to thousands, by early twenties. If patients with FAP left untreated, almost all of the colon polyps develop colorectal cancer around 40 years of age. Patients with FAP may have polyps in different organs including the upper gastrointestinal tract. However, the progression of these extra colonic polyps, to malignancy is less than colon polyps. Patients with FAP 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
Following are a few important facts about the natural history of familial adenomatous polyposis:
- Familial adenomatous polyposis is a rare disease that usually starts during teenage years.[1]
- Patients with FAP usually develop hundreds to thousands colon polyps by the age of early twenties.[2]
- Patients with FAP might be asymptomatic till third decade of their lives or whenever they have malignant transformation to colorectal cancer.
- If left untreated, almost all of the patients with FAP develop colorectal cancer around 40 years of age.
- Patients with FAP 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 include:[3][4]
- Colon cancer which has two different pathways according to defect on APC gene:
- 1- Molecular pathogenesis of sporadic colon cancer is as follows:
- 2- Molecular pathogenesis of colitis-associated colon cancer is as follows:
- 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.
- ↑ 5.0 5.1 Kim, Eun Ran (2014). "Colorectal cancer in inflammatory bowel disease: The risk, pathogenesis, prevention and diagnosis". World Journal of Gastroenterology. 20 (29): 9872. doi:10.3748/wjg.v20.i29.9872. ISSN 1007-9327.