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, 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.