Ovarian cancer in Lynch syndrome; a systematic review |
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| Affiliation: | 1. Department of Medical and Clinical Genetics, University of Helsinki, Helsinki, Finland;2. Department of Pathology, University of Helsinki and HUSLAB, Helsinki University Hospital, Finland;3. Institute for Molecular Medicine Finland, University of Helsinki, Helsinki, Finland;4. Department of Obstetrics and Gynecology, University of Helsinki, Helsinki University Hospital, Finland;5. Second Department of Surgery, Helsinki University Central Hospital, Helsinki, Finland;6. Department of Oral and Maxillofacial diseases, University of Helsinki and Helsinki University Hospital, Finland;7. Department of Surgery and Education & Science, Central Finland Health Care District, Finland;8. Department of Sport and Health Sciences, Jyväskylä University, Finland;1. Department of Medical Oncology, UT Health San Antonio, San Antonio, TX;2. Lank Center for Genitourinary Oncology, Dana-Farber Cancer Institute, Boston, MA;3. Department of Medicine, Brigham and Women''s Hospital, Harvard Medical School, Boston, MA;4. Invitae Corporation, San Francisco, CL;5. Department of Pediatrics, Division of Hematology and Oncology and Greehey Children''s Cancer Research Institute, University of Texas Health Science Center at San Antonio, San Antonio, TX;1. Department of Women''s and Children''s Health, Division of Obstetrics and Gynecology, Karolinska Institutet, Karolinska University Hospital, Solna, S-171 76 Stockholm, Sweden;2. Department of Clinical Genetics, Skåne University Hospital, 21428 Malmo-Lund, Sweden;3. Department of Clinical Pathology and Clinical Genetics, Faculty of Health Sciences, Linköping University, County Council of Östergötland, 581 85 Linköping, Sweden;4. Department of Clinical and Experimental Medicine, Faculty of Health Sciences, Linköping University, County Council of Östergötland, 581 85 Linköping, Sweden;5. Department of Clinical Genetics, Institute of Biomedicine, Sahlgrenska Academy at University of Gothenburg, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden;6. Department of Clinical Genetics, Akademiska Hospital, Uppsala University, 75185 Uppsala, Sweden;7. Department of Clinical Genetics, Karolinska Institutet, Karolinska University Hospital, Solna, 171 76 Stockholm, Sweden;8. Department of Molecular Medicine and Surgery, Karolinska Institutet, Solna, 171 76 Stockholm, Sweden;1. University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA;;2. Dana-Farber Cancer Institute, Harvard Medical School, and Brigham & Women’s Hospital, Boston, Massachusetts, USA;;3. Clinical Genetics Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York, USA;;4. Department of Medicine, Weill Cornell Medical College, New York, New York, USA. |
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| Abstract: | ObjectiveThe aim was to systematically review the characteristics of ovarian cancer in women with Lynch syndrome (LS) and evaluate the role of surveillance in detection of ovarian cancer in LS.MethodsAll studies between 1979 and 2015 of women with ovarian cancer and LS or at 50% risk of LS were evaluated. Two reviewers independently evaluated eligible studies and extracted data on age at diagnosis, histological type, FIGO stage, and way of detection according to pre-specified criteria. The studies were assessed for quality using the Newcastle-Ottawa quality assessment scales.ResultsThe quality score of the 49 identified studies was at least 6 out of 8 and provide clinical information on 747 LS women with ovarian cancer. The mean age at diagnosis was 45.3 (range 19–82) years. Most frequent mutations were MSH2 (47%) and MLH1 (38%). Histopathological data were available for 445 women. The most frequently reported histological type was mixed type (mucinous/endometrioid/clear cell carcinomas) (n = 136; 31%). Most tumours (281, 65%) were diagnosed at an early stage (FIGO I/II). Six studies evaluating the effect of surveillance of ovarian cancer, reported that seven of 22 (32%) ovarian cancers were found during surveillance, 6/7 (86%) were detected at an early stage.ConclusionThis systematic review describes that ovarian cancer in women with LS has a wide age-range of onset, is often diagnosed at an early stage with frequently endometrioid/clear cell histology. Data about the role of surveillance in detection of ovarian cancer in women with LS are scarce however detection at an early stage seems possible. |
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| Keywords: | Lynch syndrome Ovarian cancer Surveillance Screening Systematic review |
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