Peutz-Jeghers syndrome natural history, complications, and prognosis: Difference between revisions
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Prognosis is generally good if [[polypectomy]] was performed before any dysplastic degenerations take place. | Prognosis is generally good if [[polypectomy]] was performed before any dysplastic degenerations take place. | ||
==Natural History== | ==Natural History== | ||
Without [[polypectomy]], patients with Peutz-Jeghers syndrome may develop symptoms of colon cancer, which will eventually lead to death. | Without [[polypectomy]], patients with Peutz-Jeghers syndrome may develop symptoms of [[colon cancer]], which will eventually lead to death. | ||
==Complications== | ==Complications== | ||
Complications that can develop as a result of Peutz-Jeghers syndrome are:<ref name="radiopaedia">http://radiopaedia.org/articles/peutz-jeghers-syndrome-2 </ref> | Complications that can develop as a result of Peutz-Jeghers syndrome are:<ref name="radiopaedia">http://radiopaedia.org/articles/peutz-jeghers-syndrome-2 </ref> | ||
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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 if polypectomy was performed before any dysplastic degenerations take place.
Natural History
Without polypectomy, patients with Peutz-Jeghers syndrome may develop symptoms of colon cancer, which will eventually lead to death.
Complications
Complications that can develop as a result of Peutz-Jeghers syndrome are:[2]
- Colon cancer
- Cachexia
- Anemia
- Intussusception
- GI tract adenocarcinoma, although the polyps themselves are not premalignant
- Extraintestinal malignancies:
- Adenoma malignum (adenocarcinoma subtype of cervix)
- Breast cancer
- Pancreatic cancer
- Ovaries: sex cord tumors
- Testis: Sertoli cell tumors
- Lung cancer
- Uterine cancer
Prognosis
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%.[3]
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
- ↑ "eMedicine - Peutz-Jeghers Syndrome : Article by Andrea Duchini, MD". Retrieved 2007-07-21.