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Genetics of Breast and Ovarian Cancer (PDQ®)

  • Updated: 03/04/2013

Table 7. Cumulative Cancer Risks in Peutz-Jeghers Syndrome Up To Specified Agea

Site Age (y) Cumulative Risk (%)b Reference(s) 
Any cancer60–7037–93[580,582-586]
GI cancerc,d60–7038–66[582,584-586]
Gynecological cancer60–7013–18[584,586]
Per origin
Stomach6529[583]
Small bowel6513[583]
Colorectum6539[583,584]
Pancreas65–7011–36[583,584]
Lung65–707–17[583,584,586]
Breast60–7032–54[583,584,586]
Uterus659[583]
Ovary6521[583]
Cervixe6510[583]
Testese659[583]

GI = Gastrointestinal.
aReprinted with permission from Macmillan Publishers Ltd: Gastroenterology [581], copyright 2010.
bAll cumulative risks were increased compared to 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.[582]
eDid not include adenoma malignum of the cervix or Sertoli cell tumors of the testes.

References

  1. 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]

  2. 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]

  3. 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]

  4. 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]

  5. 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]

  6. 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]