Peutz-Jeghers syndrome natural history, complications, and prognosis
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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.
Complications
Complications that can develop as a result of Peutz-Jeghers syndrome are:[1][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 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%.[4]
References
- ↑ Complications of Peutz-Jeghers syndrome. Dr Amir Rezaee and Dr Alexandra Stanislavsky et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/peutz-jeghers-syndrome-2
- ↑ 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.