Surgical Pathology Criteria

Peutz-Jeghers Syndrome

Clinical

  • Mean age at presentation 24 years
  • 1/3 present ≤10 years
  • May cause bleeding or intussusception
  • Increased incidence of various cancers
  • Site Cumulative Lifetime Risk
    Overall 93%
    Stomach 21%
    Small intestine 13%
    Large intestine 34%
    Pancreas 36%
    Breast 54%
    Ovary 21%
    Cervix 10%
    Uterus 9%
    Testis 9%
    Data from Girardiello 2000, primarily based on familial cases

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