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Risk of colorectal cancer in juvenile polyposis
  1. Lodewijk A A Brosens4,
  2. Arnout van Hattem4,
  3. Linda M Hylind1,
  4. Christine Iacobuzio-Donahue3,
  5. Katharine E Romans2,
  6. Jennifer Axilbund2,
  7. Marcia Cruz-Correa5,
  8. Anne C Tersmette4,
  9. G Johan A Offerhaus4,
  10. Francis M Giardiello1
  1. 1Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  2. 2Department of Oncology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  3. 3Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  4. 4Department of Pathology, University Medical Center, Utrecht, The Netherlands
  5. 5Department of Pathology, University of Puerto Rico, San Juan, Puerto Rico
  1. Correspondence to:
    Dr F M Giardiello
    The Johns Hopkins Hospital, 1830 East Monument Street, Room 431, Baltimore, MD 21205, USA


Background: Juvenile polyposis (JP) is an autosomal-dominant syndrome characterised by the development of hamartomatous gastrointestinal polyps and is associated with colorectal cancer. However, the relative and absolute risk of colorectal malignancy in these patients is not known.

Methods: The incidence rates of colorectal cancer in patients with JP were compared with that of the general population through person-year analysis with adjustment for demographics.

Results: In patients with JP, the RR (95% CI) of colorectal cancer was 34.0 (14.4 to 65.7). Similar risks were noted in both males (30.0, 9.6 to 68.6) and females (43.7, 8.8 to 125). The cumulative life-time risk for colorectal cancer was 38.7%. The mean (SD) age of diagnosis of colorectal cancer was 43.9 (10.4) years. Other gastrointestinal malignancies were not noted in this cohort.

Conclusion: Patients with JP have a markedly increased RR and absolute risk for colorectal cancer and require vigilant colorectal surveillance starting at young age. A low threshold for recommending surgery with consideration for removal of the entire colorectum seems warranted.

  • JP, juvenile polyposis

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  • Published Online First 15 February 2007

  • Funding: This study was supported by The John G Rangos, Sr Charitable Foundation; The Clayton Fund; NIH grants CA 53801, 63721, 51085 and P50 CA 93–16; and Netherlands Digestive Disease Foundation (MLDS) grant WS 04–06.

  • Competing interests: None.

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