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Open access
Abstract
Objectives: To compare clinical–pathological characteristics
and outcome between sporadic ovarian cancer and ovarian cancer in
patents with hereditary breast and ovarian cancer syndrome (HBOC).
Methods: Twenty-four patients with ovarian cancer treated
between 2000 and 2009 who tested positive for BRCA1/2 mutation (BRCA )
and a control group of 64 age-matched patients with no family history of
breast/ovarian cancer (controls) were enrolled. Clinical–pathological
characteristics, surgical outcome, overall (OS), and progression-free
survival (PFS) were compared between the two groups.
Results: The high-grade serous histotype was more represented
in BRCA than in controls (70.8% versus 53.1%) (p > 0.05). BRCA
cancers were more frequently diagnosed at stage II than controls (20.83%
versus 4.69%) (p = 0.024). Radical primary surgery was performed in 70%
of women in both groups, with no difference in debulking results. In
patients undergoing surgery after neoadjuvant chemotherapy, in all BRCA
patients, optimal cytoreduction was achieved (versus 70% of the
controls). PFS was significantly longer for BRCA patients compared to
controls (60 months versus 22 months; p = 0.039). No significant
difference was observed in OS between BRCA patients and controls.
Conclusions: At a median follow-up time of 46 months, BRCA
patients have a better prognosis than controls in terms of PFS. Higher
chemosensitivity of BRCA tumours was observed.
Published: 03/05/2016; Received: 28/10/2015
Introduction
The estimated lifetime risk of ovarian cancer in BRCA1 mutation
carriers is 40% to 50%, among BRCA2 mutation carriers the risk is lower,
ranging from 20% to 30% [1], while the lifetime risk of ovarian cancer in the general population is 1.6%.
The mean age at diagnosis of ovarian cancer in patients with germ line
BRCA1 mutations is younger than for patients with sporadic cancer [2, 3].
Most ovarian cancers associated with hereditary breast and ovarian
cancer syndrome (HBOC) reported in the literature are high-grade and
advanced-stage serous carcinomas, whereas borderline and mucinous
tumours are uncommon [4].
Ovarian cancers in patients with HBOC are associated with unfavourable
pathological characteristics but with a higher chemosensitivity, [2, 5, 6, 7, 8, 9] as described in the term ‘BRCAness’ phenotype [10, 11]. Retrospective studies [5, 10, 11, 12, 13, 14] describe also a superior outcome; however, more recent data show only a short term but not a long-term survival advantage [15].
In the present study, we compare clinical–pathological characteristics
and outcome, in terms of overall survival (OS) and progression-free
survival (PFS), of HBOC and sporadic ovarian cancer.
Materials and methods
Study population
The study was carried out on 24 ovarian cancer patients who tested
positive for BRCA1/2 mutation (BRCA ) and 64 age-matched patients,
without any family history of breast/ovarian cancer, not tested for BRCA
mutation (controls), retrieved from the database of cases operated by
the same surgical team between 2000 and 2009 at our institution. Somatic
BRCA mutation in the tumours was not assessed.......
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