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Risk of colorectal cancer in juvenile polyposis
  1. Lodewijk A. A. Brosens (lodewijk.brosens{at}student.uva.nl)
  1. Academic Medical Center, Amsterdam, Netherlands
    1. Arnout van Hattem (vanhatten{at}hotmail.com)
    1. Academic Medical Center, Amsterdam, Netherlands
      1. Linda M. Hylind (hylinlm{at}jhmi.edu)
      1. John Hopkins University, United States
        1. Christine Iacobuzio-Donahue (ciacobu{at}jhmi.edu)
        1. John Hopkins University, United States
          1. Katharine E. Romans (romanka{at}jhmi.edu)
          1. John Hopkins University, United States
            1. Jennifer Axilbund (solleje{at}jhmi.edu)
            1. John Hopkins University, United States
              1. Marcia Cruz-Correa (marciacruz{at}rcm.upr.edu)
              1. University of Puerto Rico, Puerto Rico
                1. Anne C. Tersmette (actersmette{at}compuserve.com)
                1. University Medical Center, Utrecht, Netherlands
                  1. G. Johan A. Offerhaus (g.j.a.offerhaus{at}umcutrecht.nl)
                  1. University Medical Center, Utrecht, Netherlands
                    1. Francis M. Giardiello (fgiardi{at}jhmi.edu)
                    1. John Hopkins University, United States

                      Abstract

                      Background: Juvenile polyposis is an autosomal dominant syndrome characterized by development of hamartomatous gastrointestinal polyps and 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 juvenile polyposis patients were compared with the general population through person-year analysis with adjustment for demographics.

                      Results: In juvenile polyposis patients, the relative risk (RR) of colorectal cancer was 34.0 (95% confidence limits [CL], 14.4, 65.7). Similar risks were noted in both males (30.0, CL, 9.6, 68.6) and females (43.7, CL, 8.8-125). The cumulative life time risk for colorectal cancer was 38.7%. The mean age of colorectal cancer was 43.9+10.4 (SD). Other gastrointestinal malignancies were not noted in this cohort.

                      Conclusion: Patients with juvenile polyposis have a markedly elevated relative 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.

                      • absolute risk
                      • cancer
                      • colorectal cancer
                      • juvenile polyposis
                      • relative risk

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