@article{223cd85d3f564ebe831105909e98c7ad,
title = "Ovarian cancer linked to lynch syndrome typically presents as early-onset, non-serous epithelial tumors",
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.",
author = "Zohreh Ketabi and Katarina Bartuma and Inge Bernstein and Susanne Malander and Henrik Gr{\"o}nberg and Erik Bj{\"o}rck and Susanne Holck and Mef Nilbert",
note = "Copyright {\textcopyright} 2011 Elsevier Inc. All rights reserved.",
year = "2011",
month = jun,
doi = "10.1016/j.ygyno.2011.02.010",
language = "English",
volume = "121",
journal = "Gynecologic Oncology",
issn = "1095-6859",
publisher = "Academic Press Inc.",
number = "3",
}