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Genetics of Colorectal Cancer (PDQ®)

  • Updated: 11/12/2014

Table 13. Cumulative Cancer Risks in Peutz-Jeghers Syndrome Up To Specified Agea

Site Age (y) Cumulative Risk (%)b Reference(s) 
Any cancer60–7037–93[6,475-477,481,482]
GI cancerc,d60–7038–66[6,477,481,482]
Gynecological cancer60–7013–18[6,477]
Per origin
Stomach6529[476]
Small bowel6513[476]
Colorectum6539[6,476]
Pancreas65–7011–36[6,476]
Lung65–707–17[6,476,477]
Breast60–7032–54[6,476,477]
Uterus659[476]
Ovary6521[476]
Cervixe6510[476]
Testese659[476]

GI = gastrointestinal.
aReprinted with permission from Macmillan Publishers Ltd: Gastroenterology [478], copyright 2010.
bAll cumulative risks were increased compared with the general population (P < .05), with the exception of cervix and testes.
cGI cancers include colorectal, small intestinal, gastric, esophageal, and pancreatic.
dWesterman et al.: GI cancer does not include pancreatic cancer.[481]
eDid not include adenoma malignum of the cervix or Sertoli cell tumors of the testes.

References

  1. Hearle N, Schumacher V, Menko FH, et al.: Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res 12 (10): 3209-15, 2006.  [PUBMED Abstract]

  2. Lim W, Hearle N, Shah B, et al.: Further observations on LKB1/STK11 status and cancer risk in Peutz-Jeghers syndrome. Br J Cancer 89 (2): 308-13, 2003.  [PUBMED Abstract]

  3. Giardiello FM, Brensinger JD, Tersmette AC, et al.: Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology 119 (6): 1447-53, 2000.  [PUBMED Abstract]

  4. Lim W, Olschwang S, Keller JJ, et al.: Relative frequency and morphology of cancers in STK11 mutation carriers. Gastroenterology 126 (7): 1788-94, 2004.  [PUBMED Abstract]

  5. van Lier MG, Wagner A, Mathus-Vliegen EM, et al.: High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. Am J Gastroenterol 105 (6): 1258-64; author reply 1265, 2010.  [PUBMED Abstract]

  6. Westerman AM, Entius MM, de Baar E, et al.: Peutz-Jeghers syndrome: 78-year follow-up of the original family. Lancet 353 (9160): 1211-5, 1999.  [PUBMED Abstract]

  7. Mehenni H, Resta N, Park JG, et al.: Cancer risks in LKB1 germline mutation carriers. Gut 55 (7): 984-90, 2006.  [PUBMED Abstract]