Peutz-Jeghers syndrome natural history, complications, and prognosis: Difference between revisions
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==Natural History== | ==Natural History== | ||
If left untreated, patients with Peutz-Jeghers syndrome may progress to develop [[rectal bleeding]], [[anemia]], [[intussusception]], [[bowel obstruction]], and [[abdominal pain]].<ref name="BuckHarned1992">{{cite journal|last1=Buck|first1=J L|last2=Harned|first2=R K|last3=Lichtenstein|first3=J E|last4=Sobin|first4=L H|title=Peutz-Jeghers syndrome.|journal=RadioGraphics|volume=12|issue=2|year=1992|pages=365–378|issn=0271-5333|doi=10.1148/radiographics.12.2.1561426}}</ref> | If left untreated, patients with Peutz-Jeghers syndrome may progress to develop [[rectal bleeding]], [[anemia]], [[intussusception]], [[bowel obstruction]], and [[abdominal pain]].<ref name="BuckHarned1992">{{cite journal|last1=Buck|first1=J L|last2=Harned|first2=R K|last3=Lichtenstein|first3=J E|last4=Sobin|first4=L H|title=Peutz-Jeghers syndrome.|journal=RadioGraphics|volume=12|issue=2|year=1992|pages=365–378|issn=0271-5333|doi=10.1148/radiographics.12.2.1561426}}</ref> | ||
{| class="wikitable" | |||
|+Percentage of Gastrointestinal Cancer with Peutz-Jeghers Syndrome | |||
!Cancers | |||
!Percentage (%) | |||
|- | |||
|Colon | |||
|39 | |||
|- | |||
|Stomach | |||
|29 | |||
|- | |||
|Small Bowel | |||
|13 | |||
|- | |||
|Pancraes | |||
|11 to 39 | |||
|} | |||
==Complications== | ==Complications== | ||
Line 30: | Line 48: | ||
Prognosis is generally good with treatment. Almost half of Peutz-Jeghers patients die from cancer between age 50-60, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.<ref name="pmid2599445">{{cite journal| author=Spigelman AD, Murday V, Phillips RK| title=Cancer and the Peutz-Jeghers syndrome. | journal=Gut | year= 1989 | volume= 30 | issue= 11 | pages= 1588-90 | pmid=2599445 | doi= | pmc=PMC1434341 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2599445 }} </ref> | Prognosis is generally good with treatment. Almost half of Peutz-Jeghers patients die from cancer between age 50-60, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.<ref name="pmid2599445">{{cite journal| author=Spigelman AD, Murday V, Phillips RK| title=Cancer and the Peutz-Jeghers syndrome. | journal=Gut | year= 1989 | volume= 30 | issue= 11 | pages= 1588-90 | pmid=2599445 | doi= | pmc=PMC1434341 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2599445 }} </ref> | ||
<ref name="urlFile:PJS Natural History.jpg - Wikimedia Commons">{{cite web |url=https://commons.wikimedia.org/wiki/File:PJS_Natural_History.jpg |title=File:PJS Natural History.jpg - Wikimedia Commons |format= |work= |accessdate=}<br>Source:Wikipedia<ref name="urlFile:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology">{{cite web |url=https://librepathology.org/wiki/File:Colon_histology_with_Peutz-Jeghers_polyp.jpg |title=File:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology |format= |work= |accessdate=} | <ref name="urlFile:PJS Natural History.jpg - Wikimedia Commons"><nowiki>{{cite web |url=</nowiki>https://commons.wikimedia.org/wiki/File:PJS_Natural_History.jpg |title=File:PJS Natural History.jpg - Wikimedia Commons |format= |work= |accessdate=}<br>Source:Wikipedia<nowiki><ref name="urlFile:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology">{{cite web |url=</nowiki>https://librepathology.org/wiki/File:Colon_histology_with_Peutz-Jeghers_polyp.jpg |title=File:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology |format= |work= |accessdate=} | ||
<br clear="left"/>}</ref><br clear> | <br clear="left" />}</ref><br clear=""> | ||
[[File:PJS Natural History.jpg| | <figure-inline>[[File:PJS Natural History.jpg|960x960px]]</figure-inline>.<br> Source:Wikipedia<ref name="urlFile:PJS Natural History.jpg - Wikimedia Commons">{{cite web |url=https://commons.wikimedia.org/wiki/File:PJS_Natural_History.jpg |title=File:PJS Natural History.jpg - Wikimedia Commons |format= |work= |accessdate=}}</ref><br clear=""> | ||
== References == | == References == | ||
{{reflist|2}} | {{reflist|2}} |
Revision as of 14:36, 19 December 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]
Overview
If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain. Common complications of Peutz-Jeghers syndrome include colon cancer and cachexia. Prognosis is generally good with treatment.
Natural History
If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain.[1]
Cancers | Percentage (%) |
---|---|
Colon | 39 |
Stomach | 29 |
Small Bowel | 13 |
Pancraes | 11 to 39 |
Complications
Complications that can develop as a result of Peutz-Jeghers syndrome are:[2][3]
- Colon cancer
- Cachexia
- Anemia
- Intussusception
- Gastrointestinal tract adenocarcinoma, although the polyps themselves are not premalignant
- Extraintestinal malignancies:[4]
- Adenoma malignum (adenocarcinoma subtype of cervix)
- Breast cancer
- Pancreatic cancer
- Ovaries: sex cord tumors
- Testis: Sertoli cell tumors
- Lung cancer
- Uterine cancer
- Paraganglioma
Prognosis
Prognosis is generally good with treatment. Almost half of Peutz-Jeghers patients die from cancer between age 50-60, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.[5]
<figure-inline></figure-inline>.
Source:Wikipedia[6]
References
- ↑ Buck, J L; Harned, R K; Lichtenstein, J E; Sobin, L H (1992). "Peutz-Jeghers syndrome". RadioGraphics. 12 (2): 365–378. doi:10.1148/radiographics.12.2.1561426. ISSN 0271-5333.
- ↑ Kopacova, Marcela; Tacheci, Ilja; Rejchrt, Stanislav; Bures, Jan (2009). "Peutz-Jeghers syndrome: Diagnostic and therapeuticapproach". World Journal of Gastroenterology. 15 (43): 5397. doi:10.3748/wjg.15.5397. ISSN 1007-9327.
- ↑ Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.
- ↑ Butt N, Salih M, Khan MR, Ahmed R, Haider Z, Shah SH (2012). "An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome". Saudi J Gastroenterol. 18 (6): 388–91. doi:10.4103/1319-3767.103432. PMC 3530995. PMID 23150026.
- ↑ Spigelman AD, Murday V, Phillips RK (1989). "Cancer and the Peutz-Jeghers syndrome". Gut. 30 (11): 1588–90. PMC 1434341. PMID 2599445.
- ↑ 6.0 6.1 {{cite web |url=https://commons.wikimedia.org/wiki/File:PJS_Natural_History.jpg |title=File:PJS Natural History.jpg - Wikimedia Commons |format= |work= |accessdate=}
Source:Wikipedia<ref name="urlFile:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology">{{cite web |url=https://librepathology.org/wiki/File:Colon_histology_with_Peutz-Jeghers_polyp.jpg |title=File:Colon histology with Peutz-Jeghers polyp.jpg - Libre Pathology |format= |work= |accessdate=}
}