title: Ovarian cancer in Lynch syndrome; a systematic review.
abstract: OBJECTIVE
  The 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.
  
  
  METHODS
  All 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.
  
  
  RESULTS
  The 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.
  
  
  CONCLUSIONS
  This 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.
authors.affiliation: Department of Gynaecology, Martini Hospital, Groningen, The Netherlands; Department of Gynaecologic Oncology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands. Electronic address: j.m.woolderink@umcg.nl.
authors.forename: J M
authors.initials: JM
authors.lastname: Helder-Woolderink
authors.affiliation: Department of Gynaecologic Oncology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: E A
authors.initials: EA
authors.lastname: Blok
authors.affiliation: Department of Gastroenterology & Hepatology, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: H F A
authors.initials: HF
authors.lastname: Vasen
authors.affiliation: Department of Pathology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: Hollema
authors.affiliation: Department of Gynaecologic Oncology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Mourits
authors.affiliation: Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: G H
authors.initials: GH
authors.lastname: De Bock
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000235:genetics; D018269:Carcinoma, Endometrioid / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D055088:Early Detection of Cancer / Q000379:methods*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008875:Middle Aged; D009154:Mutation; D009367:Neoplasm Staging; D010051:Ovarian Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D010375:Pedigree; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D012307:Risk Factors; D013997:Time Factors; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: Lynch syndrome; Ovarian cancer; Screening; Surveillance; Systematic review