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Ovarian cancer linked to lynch syndrome typically presents as early-onset, non-serous epithelial tumors

Ketabi, Zohreh; Bartuma, Katarina LU ; Bernstein, Inge; Malander, Susanne LU ; Gronberg, Henrik; Bjorck, Erik; Holck, Susanne and Nilbert, Mef LU (2011) In Gynecologic Oncology 121(3). p.462-465
Abstract
Objective. Heredity is a major cause of ovarian cancer and during recent years the contribution from germline mismatch repair (MMR) gene mutations linked to Lynch syndrome has gradually been recognized. Methods. We characterized clinical features, tumor morphology and mismatch repair defects in all ovarian cancers identified in Swedish and Danish Lynch syndrome families. Results. In total, 63 epithelial ovarian cancers developed at mean 48 (range 30-79) years of age with 47% being early stage (FIGO stage I). Histologically, endometrioid (35%) and clear cell (17%) tumors were overrepresented. The underlying MMR gene mutations in these families affected MSH2 in 49%, MSH6 in 33% and MLH1 in 17%. Immunohistochemical loss of the corresponding... (More)
Objective. Heredity is a major cause of ovarian cancer and during recent years the contribution from germline mismatch repair (MMR) gene mutations linked to Lynch syndrome has gradually been recognized. Methods. We characterized clinical features, tumor morphology and mismatch repair defects in all ovarian cancers identified in Swedish and Danish Lynch syndrome families. Results. In total, 63 epithelial ovarian cancers developed at mean 48 (range 30-79) years of age with 47% being early stage (FIGO stage I). Histologically, endometrioid (35%) and clear cell (17%) tumors were overrepresented. The underlying MMR gene mutations in these families affected MSH2 in 49%, MSH6 in 33% and MLH1 in 17%. Immunohistochemical loss of the corresponding MMR protein was demonstrated in 33/36 (92%) tumors analyzed. Conclusion. The combined data from our cohorts demonstrate that ovarian cancer associated with Lynch syndrome typically presents at young age as early-stage, non-serous tumors, which implicates that a family history of colorectal and endometrial cancer should be specifically considered in such cases. (C) 2011 Elsevier Inc. All rights reserved. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
HNPCC, Ovarian tumor, MMR, Endometrioid cancer, Clear cell cancer
in
Gynecologic Oncology
volume
121
issue
3
pages
462 - 465
publisher
Academic Press
external identifiers
  • wos:000291240700007
  • scopus:79957438303
ISSN
1095-6859
DOI
10.1016/j.ygyno.2011.02.010
language
English
LU publication?
yes
id
ccb44078-0c88-44f6-ad13-c597a6417d02 (old id 1985446)
date added to LUP
2011-07-01 09:05:26
date last changed
2017-11-19 03:24:32
@article{ccb44078-0c88-44f6-ad13-c597a6417d02,
  abstract     = {Objective. Heredity is a major cause of ovarian cancer and during recent years the contribution from germline mismatch repair (MMR) gene mutations linked to Lynch syndrome has gradually been recognized. Methods. We characterized clinical features, tumor morphology and mismatch repair defects in all ovarian cancers identified in Swedish and Danish Lynch syndrome families. Results. In total, 63 epithelial ovarian cancers developed at mean 48 (range 30-79) years of age with 47% being early stage (FIGO stage I). Histologically, endometrioid (35%) and clear cell (17%) tumors were overrepresented. The underlying MMR gene mutations in these families affected MSH2 in 49%, MSH6 in 33% and MLH1 in 17%. Immunohistochemical loss of the corresponding MMR protein was demonstrated in 33/36 (92%) tumors analyzed. Conclusion. The combined data from our cohorts demonstrate that ovarian cancer associated with Lynch syndrome typically presents at young age as early-stage, non-serous tumors, which implicates that a family history of colorectal and endometrial cancer should be specifically considered in such cases. (C) 2011 Elsevier Inc. All rights reserved.},
  author       = {Ketabi, Zohreh and Bartuma, Katarina and Bernstein, Inge and Malander, Susanne and Gronberg, Henrik and Bjorck, Erik and Holck, Susanne and Nilbert, Mef},
  issn         = {1095-6859},
  keyword      = {HNPCC,Ovarian tumor,MMR,Endometrioid cancer,Clear cell cancer},
  language     = {eng},
  number       = {3},
  pages        = {462--465},
  publisher    = {Academic Press},
  series       = {Gynecologic Oncology},
  title        = {Ovarian cancer linked to lynch syndrome typically presents as early-onset, non-serous epithelial tumors},
  url          = {http://dx.doi.org/10.1016/j.ygyno.2011.02.010},
  volume       = {121},
  year         = {2011},
}