Skip to main content
NCBI home page
British Journal of Cancer logoLink to British Journal of Cancer
. 2001 Apr;84(7):969–974. doi: 10.1054/bjoc.2000.1718

Familial colorectal adenocarcinoma and hereditary nonpolyposis colorectal cancer: a nationwide epidemiological study from Sweden

K Hemminki 1, X Li 1
PMCID: PMC2363847  PMID: 11286479

Abstract

Although estimates are available of the proportion of hereditary nonpolyposis colorectal cancer (HNPCC) among all colorectal cancer (CRC), its proportion among familial CRC is unclear. We estimated these proportions epidemiologically from the nationwide Swedish Family-Cancer Database on 9.6 million individuals. Colorectal adenocarcinomas were retrieved from the Cancer Registry covering years 1958–1996. Standardized incidence ratios (SIRs) were calculated for offspring (aged less than 62 years) when their parent had colorectal adenocarcinoma. In 9.82% of all families, an offspring and a parent were affected, giving a population attributable proportion of 4.91% and a familial SIR of 2.00. When offspring and parents shared the anatomic site, the SIR was 2.32 for proximal and 2.00 for distal CRC. When offspring were diagnosed before age 40 years and parents before age 50 years, the SIR was 25.72 for familial proximal CRC. In older age groups familial risks did not differ between proximal and distal CRC. Familial risks were increased also for endometrial, small intestinal and gastric cancers, manifestations in HNPCC. Depending on which assumptions were made, HNPCC was calculated to account for 20 to 50% of familial CRC, corresponding to 1 or 2.5% of all CRC among 0–61-year-old individuals. © 2001 Cancer Research Campaign http://www.bjcancer.com

Keywords: familial risk, colorectal cancer, adenocarcinoma, endometrial cancer, right colon

Full Text

The Full Text of this article is available as a PDF (65.6 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aarnio M., Sankila R., Pukkala E., Salovaara R., Aaltonen L. A., de la Chapelle A., Peltomäki P., Mecklin J. P., Järvinen H. J. Cancer risk in mutation carriers of DNA-mismatch-repair genes. Int J Cancer. 1999 Apr 12;81(2):214–218. doi: 10.1002/(sici)1097-0215(19990412)81:2<214::aid-ijc8>3.0.co;2-l. [DOI] [PubMed] [Google Scholar]
  2. Carstensen B., Soll-Johanning H., Villadsen E., Søndergaard J. O., Lynge E. Familial aggregation of colorectal cancer in the general population. Int J Cancer. 1996 Nov 15;68(4):428–435. doi: 10.1002/(SICI)1097-0215(19961115)68:4<428::AID-IJC5>3.0.CO;2-2. [DOI] [PubMed] [Google Scholar]
  3. Evans D. G., Walsh S., Jeacock J., Robinson C., Hadfield L., Davies D. R., Kingston R. Incidence of hereditary non-polyposis colorectal cancer in a population-based study of 1137 consecutive cases of colorectal cancer. Br J Surg. 1997 Sep;84(9):1281–1285. [PubMed] [Google Scholar]
  4. Fuchs C. S., Giovannucci E. L., Colditz G. A., Hunter D. J., Speizer F. E., Willett W. C. A prospective study of family history and the risk of colorectal cancer. N Engl J Med. 1994 Dec 22;331(25):1669–1674. doi: 10.1056/NEJM199412223312501. [DOI] [PubMed] [Google Scholar]
  5. Hemminki K., Li X. Increased cancer risk in the offspring of women with colorectal carcinoma. A Swedish Register-Based Cohort Study. Cancer. 2001 Mar 1;91(5):1061–1063. [PubMed] [Google Scholar]
  6. Hemminki K., Vaittinen P., Dong C., Easton D. Sibling risks in cancer: clues to recessive or X-linked genes? Br J Cancer. 2001 Feb 2;84(3):388–391. doi: 10.1054/bjoc.2000.1585. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Hemminki K., Vaittinen P., Dong C. Endometrial cancer in the family-cancer database. Cancer Epidemiol Biomarkers Prev. 1999 Nov;8(11):1005–1010. [PubMed] [Google Scholar]
  8. Hemminki K., Vaittinen P. Familial cancers in a nationwide family cancer database: age distribution and prevalence. Eur J Cancer. 1999 Jul;35(7):1109–1117. doi: 10.1016/s0959-8049(99)00066-0. [DOI] [PubMed] [Google Scholar]
  9. Hemminki K., Vaittinen P., Kyyrönen P. Age-specific familial risks in common cancers of the offspring. Int J Cancer. 1998 Oct 5;78(2):172–175. doi: 10.1002/(sici)1097-0215(19981005)78:2<172::aid-ijc9>3.0.co;2-w. [DOI] [PubMed] [Google Scholar]
  10. Lichtenstein P., Holm N. V., Verkasalo P. K., Iliadou A., Kaprio J., Koskenvuo M., Pukkala E., Skytthe A., Hemminki K. Environmental and heritable factors in the causation of cancer--analyses of cohorts of twins from Sweden, Denmark, and Finland. N Engl J Med. 2000 Jul 13;343(2):78–85. doi: 10.1056/NEJM200007133430201. [DOI] [PubMed] [Google Scholar]
  11. Lynch H. T., Smyrk T. Hereditary nonpolyposis colorectal cancer (Lynch syndrome). An updated review. Cancer. 1996 Sep 15;78(6):1149–1167. doi: 10.1002/(SICI)1097-0142(19960915)78:6<1149::AID-CNCR1>3.0.CO;2-5. [DOI] [PubMed] [Google Scholar]
  12. Lynch H. T., de la Chapelle A. Genetic susceptibility to non-polyposis colorectal cancer. J Med Genet. 1999 Nov;36(11):801–818. [PMC free article] [PubMed] [Google Scholar]
  13. Peel D. J., Ziogas A., Fox E. A., Gildea M., Laham B., Clements E., Kolodner R. D., Anton-Culver H. Characterization of hereditary nonpolyposis colorectal cancer families from a population-based series of cases. J Natl Cancer Inst. 2000 Sep 20;92(18):1517–1522. doi: 10.1093/jnci/92.18.1517. [DOI] [PubMed] [Google Scholar]
  14. Potter J. D. Colorectal cancer: molecules and populations. J Natl Cancer Inst. 1999 Jun 2;91(11):916–932. doi: 10.1093/jnci/91.11.916. [DOI] [PubMed] [Google Scholar]
  15. Salovaara R., Loukola A., Kristo P., Käriäinen H., Ahtola H., Eskelinen M., Härkönen N., Julkunen R., Kangas E., Ojala S. Population-based molecular detection of hereditary nonpolyposis colorectal cancer. J Clin Oncol. 2000 Jun;18(11):2193–2200. doi: 10.1200/JCO.2000.18.11.2193. [DOI] [PubMed] [Google Scholar]
  16. 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 May;34(5):424–425. doi: 10.1007/BF02053699. [DOI] [PubMed] [Google Scholar]

Articles from British Journal of Cancer are provided here courtesy of Cancer Research UK

RESOURCES