Skip to main content
Log in

Clinical implications of multiple colorectal carcinomas in hereditary nonpolyposis colorectal carcinoma

  • Original Contributions
  • Published:
Diseases of the Colon & Rectum

Abstract

PURPOSE: An increased incidence of multiple (synchronous and metachronous) colorectal carcinomas has been reported in hereditary nonpolyposis colorectal cancer. This review was undertaken to determine the clinical implications of multiple colorectal carcinomas in hereditary nonpolyposis colorectal cancer. METHODS: A retrospective review of the records of patients in the hereditary nonpolyposis colorectal cancer registry at Roswell Park Cancer Institute who had either synchronous or metachronous colorectal carcinomas was conducted. RESULTS: Twenty-five of 93 patients with documented pathology were found to have multiple colorectal carcinomas. The mean age at diagnosis of the index colorectal carcinoma was 46.7 (range, 28–65) years. There were 7 (7.5 percent) patients with synchronous colorectal carcinomas and 20 (21.5 percent) patients with metachronous colorectal carcinomas. Two of the seven (28.6 percent) patients with synchronous colorectal carcinomas developed a metachronous colorectal carcinoma. In the patients with metachronous colorectal carcinomas, 29 metachronous events were noted: colon (23) and rectum (6). The mean and median time interval for metachronous colorectal carcinomas were 10.9 and 11.8 (range, 1.5–43.8) years, respectively. The mean times to first, second, and third events were 11.7 (range, 1.5–43.5), 7.9 (range, 2.7–18.7), and 12.3 (range, 11.8–12.7) years, respectively. The majority of patients with metachronous colorectal carcinomas did not have stage progression at the diagnosis of the metachronous colorectal carcinomas: 13 patients had lower or same stage at first event, 4 had lower or same stage at second event, and 2 patients had lower stage at third event. Three of 20 patients with metachronous colorectal carcinomas died of their disease. CONCLUSION: Multiple colorectal cancers are common in hereditary nonpolyposis colorectal cancer. Even though stage progression may not be evident at diagnosis of metachronous colorectal cancer, some of these patients will nevertheless die of their disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Campbell WJ, Spence RA, Parks TG. Familial adenomatous polyposis. Br J Surg 1994;81:1722–33.

    PubMed  Google Scholar 

  2. Lynch HT, Smyrk T, McGinn T,et al. Attenuated familial adenomatous polyposis (AFAP). Cancer 1995;76:2427–33.

    PubMed  Google Scholar 

  3. Desai DC, Neale KF, Talbot IC, Hodgson SV, Phillips RK. Juvenile polyposis. Br J Surg 1995;82:14–7.

    PubMed  Google Scholar 

  4. Whitelaw SC, Murday VA, Tomlinson IP,et al. Clinical and molecular features of the hereditary mixed polyposis syndrome. Gastroenterology 1997;112:327–34.

    Article  PubMed  Google Scholar 

  5. Marra G, Boland CR. Hereditary nonpolyposis colorectal cancer: the syndrome, the genes, and historical perspective. J Natl Cancer Inst 1995;87:1114–1125.

    PubMed  Google Scholar 

  6. Cannon-Albright LA, Skolnick MH, Bishop DT, Lee RG, Burt RW. Common inheritance of susceptibility to colonic adenomatous polyps and associated colorectal cancers. N Engl J Med 1988;319:533–7.

    PubMed  Google Scholar 

  7. Houlston RS, Collins A, Slack J, Morton NE. Dominant genes for colorectal cancer are not rare. Ann Hum Genet 1992;56:99–103.

    PubMed  Google Scholar 

  8. Ponz de Leon M. Descriptive epidemiology of hereditary nonpolyposis colorectal cancer. Tumori 1996;82:102–6.

    PubMed  Google Scholar 

  9. Fitzgibbons RJ, Lynch HT, Stanislav GV,et al. Recognition and treatment of patients with hereditary nonpolyposis colon cancer (Lynch syndromes I and II). Ann Surg 1987;206:289–95.

    PubMed  Google Scholar 

  10. Watson P, Lynch HT. Extracolonic cancer in hereditary nonpolyposis colorectal cancer. Cancer 1993;71:677–87.

    PubMed  Google Scholar 

  11. Lovett E. Family studies in cancer of the colon and rectum. Br J Surg 1976;63:13–8.

    PubMed  Google Scholar 

  12. Houlston RS, Murday V, Harcopos C, Williams CB, Slack J. Screening and genetic counseling for relatives of patients with colorectal cancer in a family cancer clinic. BMJ 1990;301:366–8.

    PubMed  Google Scholar 

  13. Fuchs CS, Giovannucci EL, Colditz GA,et al. A prospective study of family history and the risk of colorectal cancer. N Engl J Med 1994;331:1669–74.

    Article  PubMed  Google Scholar 

  14. Vasen HF, Mecklin JP, Khan PM, Lynch HT. The International Collaborative Group on Hereditary Nonpolyposis Colorectal Cancer (ICG-HNPCC). Dis Colon Rectum 1991;34:424–5.

    Article  PubMed  Google Scholar 

  15. Papadopoulos N, Nicolaides NC, Wei Y-S,et al. Mutation of a MutL homolog in hereditary colon cancer. Science 1994;263:1625–9.

    PubMed  Google Scholar 

  16. Leach FS, Nicolaides NC, Papadopoulos N,et al. Mutations of a MutS homolog in hereditary non-polyposis colorectal cancer. Cell 1993;75:1215–25.

    Article  PubMed  Google Scholar 

  17. Fishel R, Lescoe MK, Rao MR,et al. The mutator gene homolog MSH2 and its association with hereditary nonpolyposis colorectal cancer. Cell 1993;75:1027–38.

    Article  PubMed  Google Scholar 

  18. Bronner CE, Baker SM, Morrison PT,et al. Mutation in the DNA mismatch repair gene homologue hMLH1 is associated with hereditary nonpolyposis colorectal cancer. Nature 1994;368:258–61.

    Article  PubMed  Google Scholar 

  19. Nicolaides NC, Papadopoulos N, Wei Y-F,et al. Mutations of two PMS homologues in hereditary nonpolyposis colon cancer. Nature 1994;371:75–80.

    Article  PubMed  Google Scholar 

  20. Akiyama Y, Sato H, Yamada T,et al. Germ-line mutation of the hMSH6/GTBP gene in an atypical hereditary nonpolyposis colorectal cancer kindred. Cancer Res 1997;57:3920–23.

    PubMed  Google Scholar 

  21. Yamamoto H, Sawai H, Weber TK, Rodriguez-Bigas MA, Perucho M. Somatic frameshift mutations in DNA mismatch repair and pro-apoptosis genes in hereditary nonpolyposis colorectal cancer. Cancer Res 1998;58:997–1003.

    PubMed  Google Scholar 

  22. Liu B, Parsons R, Papadopoulos N,et al. Analysis of mismatch repair genes in hereditary nonpolyposis colorectal cancer patients. Nat Med 1996;2:169–74.

    Article  PubMed  Google Scholar 

  23. Aaltonen LA, Peltomaki P, Leach FS,et al. Clues to the pathogenesis of familial colorectal cancer. Science 1993;260:812–6.

    PubMed  Google Scholar 

  24. Mecklin JP, Järvinen HJ. Clinical features of colorectal carcinoma in cancer family syndrome. Dis Colon Rectum 1986;29:160–4.

    PubMed  Google Scholar 

  25. Colon and Rectum. In: Fleming ID, Cooper JS, Henson DE,et al., eds. American Joint Committee on Cancer: Cancer Staging Manual. 5th ed. Philadelphia, New York: Lippincott-Raven, 1997:183–8.

    Google Scholar 

  26. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc 1958;53:457–86.

    Google Scholar 

  27. Cox DR. Regression models and life tables. J R Stat Soc 1972;34:187–220.

    Google Scholar 

  28. Vasen HF, Wijnen JT, Menko FH,et al. Cancer risk in families with hereditary nonpolyposis colorectal cancer diagnosed by mutation analysis. Gastroenterology 1996;110:1020–7.

    Article  PubMed  Google Scholar 

  29. Watson P, Lin K, Rodriguez-Bigas MA,et al. Colorectal cancer survival in hereditary colorectal cancer family members. Cancer 1998;83:259–66.

    Article  PubMed  Google Scholar 

  30. Fante R, Roncucci L, di Gregorio C,et al. Frequency and clinical features of multiple tumors of the large bowel in the general population and in patients with hereditary colorectal carcinoma. Cancer 1996;77:2013–21.

    Article  PubMed  Google Scholar 

  31. Slater G, Aufses AH, Szporn A. Synchronous carcinoma of the colon and rectum. Surg Gynecol Obstet 1990;171:283–7.

    PubMed  Google Scholar 

  32. Rodriguez-Bigas MA, Vasen HF, Pekka-Mecklin J,et al. and the International Collaborative Group on HNPCC. Rectal cancer risk in hereditary nonpolyposis colorectal cancer after total abdominal colectomy. Ann Surg 1997;225:202–7.

    Article  PubMed  Google Scholar 

  33. Vasen HFA, Nagengast FM, Meera Khan P. Interval cancers in hereditary non-polyposis colorectal cancer (Lynch syndrome). Lancet 1995;345:1183–4.

    Article  PubMed  Google Scholar 

  34. Byers, T, Levin B, Rothenberger D, Dodd GD, Smith RA. American Cancer Society guidelines for screening and surveillance for early detection of colorectal polyps and cancer: update 1997. American Cancer Society Detection and Treatment Advisory Group on Colorectal Cancer. CA Cancer J Clin 1997;47:154–60.

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

About this article

Cite this article

Box, J.C., Rodriguez-Bigas, M.A., Weber, T.K. et al. Clinical implications of multiple colorectal carcinomas in hereditary nonpolyposis colorectal carcinoma. Dis Colon Rectum 42, 717–721 (1999). https://doi.org/10.1007/BF02236924

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02236924

Key words

Navigation