Peutz-Jeghers syndrome natural history, complications, and prognosis: Difference between revisions
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__NOTOC__ | __NOTOC__ | ||
{{Peutz-Jeghers syndrome}} | {{Peutz-Jeghers syndrome}} | ||
{{CMG}} {{AE}} {{HQ}} | {{CMG}}; {{AE}} {{HQ}} | ||
==Overview== | ==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]]. | 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. | ||
Prognosis is generally good with treatment. | |||
==Natural History | ==Natural History, Complications, and Prognosis== | ||
{| | === Natural history === | ||
|+Percentage of | 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><ref name="GiardielloTrimbath2006">{{cite journal|last1=Giardiello|first1=F|last2=Trimbath|first2=J|title=Peutz-Jeghers Syndrome and Management Recommendations|journal=Clinical Gastroenterology and Hepatology|volume=4|issue=4|year=2006|pages=408–415|issn=15423565|doi=10.1016/j.cgh.2005.11.005}}</ref><ref name="pmid20051941">{{cite journal| author=van Lier MG, Wagner A, Mathus-Vliegen EM, Kuipers EJ, Steyerberg EW, van Leerdam ME| title=High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. | journal=Am J Gastroenterol | year= 2010 | volume= 105 | issue= 6 | pages= 1258-64; author reply 1265 | pmid=20051941 | doi=10.1038/ajg.2009.725 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20051941 }} </ref> | ||
!Cancers | |||
!Percentage (%) | {| | ||
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Percentage of extraintestinal cancer in Peutz-Jeghers syndrome | |||
|- | |||
! style="background:#7d7d7d; color: #FFFFFF;" align="center" + |Cancers | |||
! style="background:#7d7d7d; color: #FFFFFF;" align="center" + |Percentage (%) | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Breast | |||
| style="background:#F5F5F5;" align="center" + |32 to 54 | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Ovary | |||
| style="background:#F5F5F5;" align="center" + |21 | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Cervix | |||
| style="background:#F5F5F5;" align="center" + |10 | |||
|- | |||
| style="background:#DCDCDC;" align="center" + |Testes | |||
| style="background:#F5F5F5;" align="center" + |9 | |||
|} | |||
<br> | |||
{| | |||
! colspan="2" style="background:#4479BA; color: #FFFFFF;" align="center" + |Percentage of gastrointestinal cancer in Peutz-Jeghers syndrome | |||
|- | |- | ||
| | ! style="background:#7d7d7d; color: #FFFFFF;" align="center" + |Cancers | ||
| | ! style="background:#7d7d7d; color: #FFFFFF;" align="center" + |Percentage (%) | ||
|- | |- | ||
| | | style="background:#DCDCDC;" align="center" + |Colon | ||
| | | style="background:#F5F5F5;" align="center" + |39 | ||
|- | |- | ||
| | | style="background:#DCDCDC;" align="center" + |Stomach | ||
| | | style="background:#F5F5F5;" align="center" + |29 | ||
|- | |- | ||
|Pancraes | | style="background:#DCDCDC;" align="center" + |Small Bowel | ||
|11 to 39 | | style="background:#F5F5F5;" align="center" + |13 | ||
|- | |||
| style="background:#DCDCDC;" align="center" + |Pancraes | |||
| style="background:#F5F5F5;" align="center" + |11 to 39 | |||
|} | |} | ||
==Complications== | ===Complications=== | ||
Complications that can develop as a result of Peutz-Jeghers syndrome are:<ref name="KopacovaTacheci2009">{{cite journal|last1=Kopacova|first1=Marcela|last2=Tacheci|first2=Ilja|last3=Rejchrt|first3=Stanislav|last4=Bures|first4=Jan|title=Peutz-Jeghers syndrome: Diagnostic and therapeuticapproach|journal=World Journal of Gastroenterology|volume=15|issue=43|year=2009|pages=5397|issn=1007-9327|doi=10.3748/wjg.15.5397}}</ref><ref>Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.</ref> | Complications that can develop as a result of Peutz-Jeghers syndrome are:<ref name="KopacovaTacheci2009">{{cite journal|last1=Kopacova|first1=Marcela|last2=Tacheci|first2=Ilja|last3=Rejchrt|first3=Stanislav|last4=Bures|first4=Jan|title=Peutz-Jeghers syndrome: Diagnostic and therapeuticapproach|journal=World Journal of Gastroenterology|volume=15|issue=43|year=2009|pages=5397|issn=1007-9327|doi=10.3748/wjg.15.5397}}</ref><ref>Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.</ref><ref name="pmid23150026">{{cite journal| author=Butt N, Salih M, Khan MR, Ahmed R, Haider Z, Shah SH| title=An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome. | journal=Saudi J Gastroenterol | year= 2012 | volume= 18 | issue= 6 | pages= 388-91 | pmid=23150026 | doi=10.4103/1319-3767.103432 | pmc=PMC3530995 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23150026 }} </ref> | ||
*[[Colon cancer]] | *[[Colon cancer]] | ||
*[[Cachexia]] | *[[Cachexia]] | ||
*[[Anemia]] | *[[Anemia]] | ||
*[[Intussusception]] | *[[Intussusception]] | ||
*[[Gastrointestinal tract]] [[adenocarcinoma]], although the polyps themselves are not premalignant | *[[Gastrointestinal tract]] [[adenocarcinoma]], although the polyps themselves are not [[premalignant]] | ||
* | |||
:*Adenoma malignum (adenocarcinoma subtype of cervix) | * Extra-intestinal [[malignancies]]: | ||
:*[[Adenoma]] malignum ([[adenocarcinoma]] subtype of [[cervix]]) | |||
:*[[Breast cancer]] | :*[[Breast cancer]] | ||
:*[[Pancreatic cancer]] | :*[[Pancreatic cancer]] | ||
:*[[Ovaries]]: [[sex cord]] | :*[[Ovaries]]: [[Sex cord-stromal tumor|sex cord tumors]] | ||
:*[[Testis]]: [[Sertoli cell]] tumors | :*[[Testis]]: [[Sertoli cell]] [[tumors]] | ||
:*[[Lung cancer]] | :*[[Lung cancer]] | ||
:*[[Uterine cancer]] | :*[[Uterine cancer]] | ||
:*[[Paraganglioma]] | :*[[Paraganglioma]] | ||
==Prognosis== | ===Prognosis=== | ||
Prognosis is generally good with treatment | *Prognosis is generally good with treatment.<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><br clear=""> | ||
*Almost half of Peutz-Jeghers patients die from [[cancer]] between age 50-60 years. | |||
< | *The cumulative risk of developing a form of [[cancer]] associated with Peutz-Jeghers syndrome between ages 15-64 is 93%. | ||
== References == | == References == | ||
{{reflist|2}} | {{reflist|2}} | ||
{{WH}} | |||
{{WS}} | |||
[[Category:Gastroenterology]] | |||
[[Category:Surgery]] | |||
[[Category:Oncology]] | |||
[[Category:Disease]] | |||
[[Category:Pediatrics]] | |||
[[Category:Up-To-Date]] |
Latest revision as of 16:44, 21 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, Complications, and Prognosis
Natural history
If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain.[1][2][3]
Percentage of extraintestinal cancer in Peutz-Jeghers syndrome | |
---|---|
Cancers | Percentage (%) |
Breast | 32 to 54 |
Ovary | 21 |
Cervix | 10 |
Testes | 9 |
Percentage of gastrointestinal cancer in Peutz-Jeghers syndrome | |
---|---|
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:[4][5][6]
- Colon cancer
- Cachexia
- Anemia
- Intussusception
- Gastrointestinal tract adenocarcinoma, although the polyps themselves are not premalignant
- Extra-intestinal malignancies:
- 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.[7]
- Almost half of Peutz-Jeghers patients die from cancer between age 50-60 years.
- The cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.
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.
- ↑ Giardiello, F; Trimbath, J (2006). "Peutz-Jeghers Syndrome and Management Recommendations". Clinical Gastroenterology and Hepatology. 4 (4): 408–415. doi:10.1016/j.cgh.2005.11.005. ISSN 1542-3565.
- ↑ van Lier MG, Wagner A, Mathus-Vliegen EM, Kuipers EJ, Steyerberg EW, van Leerdam ME (2010). "High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations". Am J Gastroenterol. 105 (6): 1258–64, author reply 1265. doi:10.1038/ajg.2009.725. PMID 20051941.
- ↑ 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.