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Risk of gynecologic cancers in Danish hereditary non-polyposis colorectal cancer families.
Acta Obstet Gynecol Scand 2008; 87(11):1129-35AO

Abstract

OBJECTIVE

Women in hereditary non-polyposis colorectal cancer (HNPCC) families have an elevated risk of endometrial and ovarian cancer. The risk in Lynch syndrome families with known mutations in mismatch repair genes (MMR genes) seems to be higher than in familial colorectal cancer (CRC) families. Data in the Danish HNPCC register on the frequency and lifetime risk of gynecologic cancers were analyzed and the actual surveillance strategy discussed in relation to the results.

DESIGN

Register-based retrospective study.

METHOD

A total of 1,780 at-risk women were identified and epidemiological, clinical and MMR gene mutation data were retrieved.

RESULTS

In a total of 105 cases of endometrial cancer, there was no significant difference in MSH2, MSH6 and MLH1 mutation carrier frequency. Compared to the general population, mutation carriers had a 20 times increase in lifetime risk of endometrial cancer. Lifetime risk was elevated four times in familial CRC families. In these families, frequency was correlated to the pedigree phenotype, with significantly higher frequency demonstrated in Amsterdam II families compared to Amsterdam I families and families suspected of HNPCC. A total of 39 cases of ovarian cancer were identified with a lifetime risk of three to four times the general population. No significant correlation was found between the frequency of ovarian cancer and MMR gene mutation status in the families.

CONCLUSION

The benefit of surveillance concerning gynecological cancers seems to be less well founded in familial CRC families than in Lynch syndrome families. Modifying the surveillance strategy may be relevant in the future, but before changing existing guidelines concerning surveillance, further research is recommended.

Authors+Show Affiliations

The Danish HNPCC Register, Department of Gastroenterology 435, Hvidovre University Hospital, Hvidovre, Denmark. a.boile@dadlnet.dkNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

18972272

Citation

Boilesen, Astrid Elisabeth Bruun, et al. "Risk of Gynecologic Cancers in Danish Hereditary Non-polyposis Colorectal Cancer Families." Acta Obstetricia Et Gynecologica Scandinavica, vol. 87, no. 11, 2008, pp. 1129-35.
Boilesen AE, Bisgaard ML, Bernstein I. Risk of gynecologic cancers in Danish hereditary non-polyposis colorectal cancer families. Acta Obstet Gynecol Scand. 2008;87(11):1129-35.
Boilesen, A. E., Bisgaard, M. L., & Bernstein, I. (2008). Risk of gynecologic cancers in Danish hereditary non-polyposis colorectal cancer families. Acta Obstetricia Et Gynecologica Scandinavica, 87(11), pp. 1129-35. doi:10.1080/00016340802443806.
Boilesen AE, Bisgaard ML, Bernstein I. Risk of Gynecologic Cancers in Danish Hereditary Non-polyposis Colorectal Cancer Families. Acta Obstet Gynecol Scand. 2008;87(11):1129-35. PubMed PMID: 18972272.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Risk of gynecologic cancers in Danish hereditary non-polyposis colorectal cancer families. AU - Boilesen,Astrid Elisabeth Bruun, AU - Bisgaard,Marie Luise, AU - Bernstein,Inge, PY - 2008/10/31/pubmed PY - 2008/12/30/medline PY - 2008/10/31/entrez SP - 1129 EP - 35 JF - Acta obstetricia et gynecologica Scandinavica JO - Acta Obstet Gynecol Scand VL - 87 IS - 11 N2 - OBJECTIVE: Women in hereditary non-polyposis colorectal cancer (HNPCC) families have an elevated risk of endometrial and ovarian cancer. The risk in Lynch syndrome families with known mutations in mismatch repair genes (MMR genes) seems to be higher than in familial colorectal cancer (CRC) families. Data in the Danish HNPCC register on the frequency and lifetime risk of gynecologic cancers were analyzed and the actual surveillance strategy discussed in relation to the results. DESIGN: Register-based retrospective study. METHOD: A total of 1,780 at-risk women were identified and epidemiological, clinical and MMR gene mutation data were retrieved. RESULTS: In a total of 105 cases of endometrial cancer, there was no significant difference in MSH2, MSH6 and MLH1 mutation carrier frequency. Compared to the general population, mutation carriers had a 20 times increase in lifetime risk of endometrial cancer. Lifetime risk was elevated four times in familial CRC families. In these families, frequency was correlated to the pedigree phenotype, with significantly higher frequency demonstrated in Amsterdam II families compared to Amsterdam I families and families suspected of HNPCC. A total of 39 cases of ovarian cancer were identified with a lifetime risk of three to four times the general population. No significant correlation was found between the frequency of ovarian cancer and MMR gene mutation status in the families. CONCLUSION: The benefit of surveillance concerning gynecological cancers seems to be less well founded in familial CRC families than in Lynch syndrome families. Modifying the surveillance strategy may be relevant in the future, but before changing existing guidelines concerning surveillance, further research is recommended. SN - 1600-0412 UR - https://www.unboundmedicine.com/medline/citation/18972272/Risk_of_gynecologic_cancers_in_Danish_hereditary_non_polyposis_colorectal_cancer_families_ L2 - https://doi.org/10.1080/00016340802443806 DB - PRIME DP - Unbound Medicine ER -