Characteristics of Lynch syndrome associated ovarian cancer

To describe clinical characteristics of Lynch syndrome associated ovarian cancer and the efficacy of surveillance in the early detection of these ovarian cancers. All Lynch syndrome associated ovarian cancer cases identified in either the Dutch Lynch syndrome registry (DLSR) between 1987 and 2016, a...

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Published in:Gynecologic oncology 2018-08, Vol.150 (2), p.324-330
Main Authors: Woolderink, J.M., De Bock, G.H., de Hullu, J.A., Hollema, H., Zweemer, R.P., Slangen, B.F.M., Gaarenstroom, K.N., van Beurden, M., van Doorn, H.C., Sijmons, R.H., Vasen, H.F.A., Mourits, M.J.E.
Format: Article
Language:English
Subjects:
Lynch syndrome
Ovarian cancer
Surveillance
Survival
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Summary:To describe clinical characteristics of Lynch syndrome associated ovarian cancer and the efficacy of surveillance in the early detection of these ovarian cancers. All Lynch syndrome associated ovarian cancer cases identified in either the Dutch Lynch syndrome registry (DLSR) between 1987 and 2016, and/or the cohort at the University Medical Center Groningen (UMCG) between 1993 and 2016 were included. Clinical data on age at diagnosis, mutation type, histological type, FIGO stage, treatment, follow-up and gynecological surveillance were collected. A total of 46/798 (6%) women in the DLSR and 7/80 (9%) in the UMCG cohort were identified as LS associated ovarian cancer patients. The median age at ovarian cancer diagnosis was 46.0 years (range 20–75 years). The most frequently reported histological type was endometrioid adenocarcinoma (40%; n = 21) and serous carcinoma (36%; n = 19). Most tumors (87%; n = 46) were detected at an early stage (FIGO I/II). Forty-one of 53 (77%) patients were diagnosed with ovarian cancer before LS was diagnosed. In the other 12/53 (23%) women, ovarian cancer developed after starting annual gynecological surveillance for LS; three ovarian cancers were screen-detected in asymptomatic women. Overall survival was 83%. Ovarian cancer in women with LS has a wide age-range of onset, is usually diagnosed at an early stage with predominantly endometrioid type histology and a good overall survival. The early stage at diagnosis could not be attributed to annual gynecological surveillance. •Ovarian cancer in women with LS develops at an early age, with a wide age-range of onset and at an early stage.•These LS associated ovarian cancers most often has an endometrioid or serous type histology with a good overall survival.•The early stage of ovarian cancer, in women with LS, could not be attributed to annual gynecological surveillance.
ISSN:0090-8258
1095-6859
DOI:10.1016/j.ygyno.2018.03.060