The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome

Patrice Watson, H. F A Vasen, Jukka Pekka Mecklin, Inge Bernstein, Markku Aarnio, Heikki J. Järvinen, Torben Myrhoj, Lone Sunde, Juul T. Wijnen, Henry T. Lynch

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Abstract

Persons with the Lynch syndrome (LS) are at high risk for cancer, including cancers of the small bowel, stomach, upper urologic tract (renal pelvis and ureter), ovary, biliary tract and brain tumors, in addition to the more commonly observed colorectal and endometrial cancers. Cancer prevention strategies for these less common cancers require accurate, age-specific risk estimation. We pooled data from 4 LS research centers in a retrospective cohort study, to produce absolute incidence estimates for these cancer types, and to evaluate several potential risk modifiers. After elimination of 135 persons missing crucial information, cohort included 6,041 members of 261 families with LS-associated MLH1 or MSH2 mutations. All were either mutation carriers by test, probable mutation carriers (endometrial/colorectal cancer-affected), or first-degree relatives of these. Among mutation carriers and probable carriers, urologic tract cancer (N = 98) had an overall lifetime risk (to age 70) of 8.4% (95% CI: 6.6-10.8); risks were higher in males (p <0.02) and members of MSH2 families (p <0.0001). Ovarian cancer (N = 72) had an lifetime risk of 6.7% (95% CI: 5.3-9.1); risks were higher in women born after the median year of birth (p <0.008) and in members of MSH2 families (p <0.006). Brain tumors and cancers of the small bowel, stomach, breast and biliary tract were less common. Urologic tract cancer and ovarian cancer occur frequently enough in some LS subgroups to justify trials to evaluate promising prevention interventions. Other cancer types studied occur too infrequently to justify strenuous cancer control interventions.

Original languageEnglish
Pages (from-to)444-449
Number of pages6
JournalInternational Journal of Cancer
Volume123
Issue number2
DOIs
StatePublished - Jul 15 2008

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Hereditary Nonpolyposis Colorectal Neoplasms
Endometrial Neoplasms
Brain Neoplasms
Urologic Neoplasms
Intestinal Neoplasms
Neoplasms
Mutation
Biliary Tract
Ovarian Neoplasms
Colorectal Neoplasms
Stomach
Kidney Pelvis
Ureter
Ovary
Breast
Cohort Studies
Retrospective Studies
Parturition
Incidence
Research

All Science Journal Classification (ASJC) codes

  • Cancer Research
  • Medicine(all)
  • Oncology

Cite this

Watson, P., Vasen, H. F. A., Mecklin, J. P., Bernstein, I., Aarnio, M., Järvinen, H. J., ... Lynch, H. T. (2008). The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome. International Journal of Cancer, 123(2), 444-449. https://doi.org/10.1002/ijc.23508

The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome. / Watson, Patrice; Vasen, H. F A; Mecklin, Jukka Pekka; Bernstein, Inge; Aarnio, Markku; Järvinen, Heikki J.; Myrhoj, Torben; Sunde, Lone; Wijnen, Juul T.; Lynch, Henry T.

In: International Journal of Cancer, Vol. 123, No. 2, 15.07.2008, p. 444-449.

Research output: Contribution to journalArticle

Watson, P, Vasen, HFA, Mecklin, JP, Bernstein, I, Aarnio, M, Järvinen, HJ, Myrhoj, T, Sunde, L, Wijnen, JT & Lynch, HT 2008, 'The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome', International Journal of Cancer, vol. 123, no. 2, pp. 444-449. https://doi.org/10.1002/ijc.23508
Watson P, Vasen HFA, Mecklin JP, Bernstein I, Aarnio M, Järvinen HJ et al. The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome. International Journal of Cancer. 2008 Jul 15;123(2):444-449. https://doi.org/10.1002/ijc.23508
Watson, Patrice ; Vasen, H. F A ; Mecklin, Jukka Pekka ; Bernstein, Inge ; Aarnio, Markku ; Järvinen, Heikki J. ; Myrhoj, Torben ; Sunde, Lone ; Wijnen, Juul T. ; Lynch, Henry T. / The risk of extra-colonic, extra-endometrial cancer in the Lynch syndrome. In: International Journal of Cancer. 2008 ; Vol. 123, No. 2. pp. 444-449.
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