Table 9. Cumulative Cancer Risks in Peutz-Jeghers Syndrome Up To Specified Agea

SiteAge (y)Cumulative Risk (%)bReference(s)
Any cancer60–7037–93[392-395,399,400]
GI cancerc,d60–7038–66[394,395,399,400]
Gynecological cancer60–7013–18[394,395]
Per origin
Stomach6529[393]
Small bowel6513[393]
Colorectum6539[393,394]
Pancreas65–7011–36[393,394]
Lung65–707–17[393-395]
Breast60–7032–54[393-395]
Uterus659[393]
Ovary6521[393]
Cervixe6510[393]
Testese659[393]

GI = gastrointestinal.

aReprinted with permission from Macmillan Publishers Ltd: Gastroenterology [396], 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.[399]

eDid not include adenoma malignum of the cervix or Sertoli cell tumors of the testes.

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