Peutz-Jeghers syndrome natural history, complications, and prognosis: Difference between revisions
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*[[Anemia]] | *[[Anemia]] | ||
*[[Intussusception]] | *[[Intussusception]] | ||
* | *[[Gastrointestinal tract]] [[adenocarcinoma]], although the polyps themselves are not premalignant | ||
*Extraintestinal malignancies:<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> | *Extraintestinal malignancies:<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> | ||
:*Adenoma malignum (adenocarcinoma subtype of cervix) | :*Adenoma malignum (adenocarcinoma subtype of cervix) | ||
Revision as of 14:40, 21 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [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.[1] 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.
Complications
Complications that can develop as a result of Peutz-Jeghers syndrome are:[2]
- Colon cancer
- Cachexia
- Anemia
- Intussusception
- Gastrointestinal tract adenocarcinoma, although the polyps themselves are not premalignant
- Extraintestinal malignancies:[3]
- 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 by age 57 years, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.[4]
References
- ↑ Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.
- ↑ http://radiopaedia.org/articles/peutz-jeghers-syndrome-2
- ↑ 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.
- ↑ "eMedicine - Peutz-Jeghers Syndrome : Article by Andrea Duchini, MD". Retrieved 2007-07-21.