Peutz-Jeghers syndrome natural history, complications, and prognosis: Difference between revisions
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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=} | |||
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[[File:PJS Natural History.jpg|300px|Development of Cancer]].<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 20:22, 18 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]
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]
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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.
- ↑ "File:PJS Natural History.jpg - Wikimedia Commons".
