Highlights
- •In this systematic review 747 ovarian cancers in women with LS were evaluated.
- •The mean age of diagnosis of ovarian cancer was 45.3 years (range 19–82 years).
- •Most common histological types were endometrioid or clear cell carcinomas.
- •65% of ovarian cancers were early stage (FIGO I/II) with a good overall survival.
- •In six studies, 7/22 (32%) ovarian cancers were found during surveillance.
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.
Conclusion
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.
Keywords
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Article info
Publication history
Published online: January 13, 2016
Accepted:
December 6,
2015
Received in revised form:
December 1,
2015
Received:
July 7,
2015
Identification
Copyright
© 2015 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.