Familial Colorectal Cancer Type X



Three to four percent of colon cancers occur in the setting of inherited or familial cancer syndrome. Currently approximately half of these are currently attributed to hereditary nonpolyposis colorectal cancer (HNPCC) [1, 2].


Lynch Syndrome Amsterdam Criterion Lynch Syndrome Patient Bethesda Criterion Sessile Serrate Adenoma 
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  1. 1.
    Brand, R.M., et al. Risk of colon cancer in hereditary non-polyposis colorectal cancer patients as predicted by fuzzy modeling: influence of smoking. World J Gastroenterol 2006. 12(28):4485–91.PubMedGoogle Scholar
  2. 2.
    Boland, C.R. Decoding hereditary colorectal cancer. N Engl J Med 2006. 354(26):2815–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Lynch, H.T., Lynch, P.M., Albano, W.A., Lynch, J.F. The cancer family syndrome: a status report. Dis Colon Rectum 1981. 24:311–22.PubMedCrossRefGoogle Scholar
  4. 4.
    Lynch, H.T., et al. Hereditary nonpolyposis colorectal cancer (Lynch syndromes I and II). II. Biomarker studies. Cancer 1985. 56:939–51.PubMedCrossRefGoogle Scholar
  5. 5.
    Muir, G.G., Bell, A.Y., Barlow, K.A. Multiple primary carcinomata of the colon, duodenum, and larynx associated with keratoacanthomata of the face. Br J Surg 1967. 54:191–5.PubMedCrossRefGoogle Scholar
  6. 6.
    Torre, D. Multiple sebaceous tumors. Arch Derm 1968. 98:549–51.PubMedCrossRefGoogle Scholar
  7. 7.
    Jass, J.R. Hereditary non-polyposis colorectal cancer: the rise and fall of a confusing term. World J Gastroenterol 2006. 12(31):4943–50.PubMedGoogle Scholar
  8. 8.
    Jass, J.R., et al. Pathology of hereditary non-polyposis colorectal cancer. Anticancer Res 1994. 14(4B):1631–4.PubMedGoogle Scholar
  9. 9.
    Sarroca, C., et al. Three new mutations in hereditary nonpolyposis colorectal cancer (Lynch syndrome II) in Uruguay. Cancer Genet Cytogenet 2003. 142(1):13–20.PubMedCrossRefGoogle Scholar
  10. 10.
    Wagner, A., et al. Molecular analysis of hereditary nonpolyposis colorectal cancer in the United States: high mutation detection rate among clinically selected families and characterization of an American founder genomic deletion of the MSH2 gene. Am J Hum Genet 2003. 72(5):1088–100.PubMedCrossRefGoogle Scholar
  11. 11.
    Wijnen, J., et al. MSH2 genomic deletions are a frequent cause of HNPCC. Nat Genet 1998. 20(4):326–8.PubMedCrossRefGoogle Scholar
  12. 12.
    Vasen, H.F., Mecklin, J.P., Khan, P.M., Lynch, H.T. The International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer (ICG-HNPCC). Dis Colon Rectum 1991. 34:424–5.PubMedCrossRefGoogle Scholar
  13. 13.
    Vasen, H.F., Watson, P., Mecklin, J.P., Lynch, H.T. New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the INternaltional Collaborative group on HNPCC. Gastroenterology 1999. 116:1453–6.PubMedCrossRefGoogle Scholar
  14. 14.
    Umar, A., et al. Revised Bethesda Guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability. J Natl Cancer Inst 2004. 96(4):261–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Lindor, N.M., et al. Lower cancer incidence in Amsterdam-I criteria families without mismatch repair deficiency: familial colorectal cancer type X. JAMA 2005. 293(16):1979–85.PubMedCrossRefGoogle Scholar
  16. 16.
    Wijnen, J.T., et al. Clinical findings with implications for genetic testing in families with clustering of colorectal cancer. N Engl J Med 1998. 339(8):511–8.PubMedCrossRefGoogle Scholar
  17. 17.
    Mueller-Koch, Y., et al. Hereditary non-polyposis colorectal cancer: clinical and molecular evidence for a new entity of hereditary colorectal cancer. Gut 2005. 54(12):1733–40.PubMedCrossRefGoogle Scholar
  18. 18.
    Lindor, N.M., et al. Recommendations for the care of individuals with an inherited predisposition to Lynch syndrome: a systematic review. JAMA 2006. 296(12):1507–17.PubMedCrossRefGoogle Scholar
  19. 19.
    Smith, R.A. et al. American Cancer Society. American Cancer Society guidelines for the early detection of cancer. Cancer J Clin 2004. 54:41–52.CrossRefGoogle Scholar
  20. 20.
    National Comprehensive Cancer Network. National Comprehensive Cancer Network Practice Guidelines in Oncology, 2004.Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Division of Colon and Rectal Surgery, Beth Israel Deaconess Medical CenterHarvard UniversityBostonUSA

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