What are the cancer surveillance recommendations in Peutz-Jeghers syndrome (PJS)?

Updated: Oct 11, 2018
  • Author: Buu Anh T To, MD; Chief Editor: Praveen K Roy, MD, AGAF  more...
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A review by Beggs and coworkers summarized recommendations for cancer surveillance in patients with Peutz-Jeghers syndrome and pooled some published recommendations. [112, 113, 114] Recommendations were based on literature review, cohort studies, and systematic review. Recommendations focused on upper intestinal, colorectal, pancreatic, breast, and genitourinary (reproductive) organ surveillance.

  • Upper gastrointestinal tract: Annual hemoglobin concentration, esophagogastroduodenoscopy (EGD) every 2-3 years, small bowel series/enteroscopy (possible alternatives: wireless capsule endoscopy, magnetic resonance enterography) every 2 years, although there is no consensus on from what age (eg, age 10, 18, 25 years) to start

  • Colorectal: Colonoscopy or flexible sigmoidoscopy and barium enema every 2-3 years from the time of first symptoms or in late teens or age 25 years onward

  • Pancreatic: Annual abdominal ultrasonography or annual/every-other-year endoscopic ultrasonography from age 25-30 years onward

  • Breast: Regular breast examination (monthly to 6-monthly) from age 18 years onward, mammography (or magnetic resonance imaging [MRI]) every other year (annually after age 50 years)

  • Genitourinary in women: Annual pelvic examination, pelvic ultrasonography, and cervical smears; some reviews recommend serum CA-125, endometrial biopsy annually from age 20 years onward

  • Genitourinary in men: Annual testicular examination; ultrasonography if symptomatic from birth

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