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Familial cancer among consecutive uterine cancer patients in Sweden.
[cowden syndrome]
Uterine
cancer
(
UC
)
represents
5
.
1
%
of
all
female
malignancies
in
Sweden
.
Accumulation
of
UC
in
families
occurs
in
around
5
%
of
cases
.
We
wanted
to
identify
any
familial
association
between
UC
and
other
selected
cancers
and
to
study
the
frequency
of
Lynch
,
Cowden
and
cancer
syndromes
among
consecutive
UC
patients
in
Sweden
.
481
UC
patients
were
included
.
Information
on
the
cancer
diagnoses
of
their
relatives
(
first
-
(
FDRs
)
and
second
-degree
(
SDRs
)
relatives
and
first
cousins
)
was
obtained
.
The
relative
frequencies
of
different
cancers
among
relatives
were
compared
to
those
in
the
Swedish
general
cancer
population
in
1970
and
2010
.
Families
that
fulfilled
the
criteria
for
hereditary
cancer
syndromes
were
tested
for
mutations
in
the
causative
genes
.
Families
with
at
least
one
case
of
UC
in
addition
to
the
index
patient
were
compared
to
families
with
no
additional
cases
to
investigate
possible
characteristics
of
putative
hereditary
cancer
syndromes
.
There
was
an
increased
prevalence
of
UC
in
our
study
population
compared
to
the
Swedish
general
cancer
population
in
1970
and
2010
(
6
%
vs
.
4
%
and
3
%
,
respectively
)
.
Seven
families
had
Lynch
Syndrome
according
to
the
Amsterdam
II
criteria
.
No
families
fulfilled
the
criteria
for
Cowden
syndrome
.
In
total
13
%
of
index
patients
had
at
least
one
relative
with
UC
and
these
families
tended
to
have
more
cases
of
early
onset
cancer
among
family
members
.
In
addition
,
16
%
of
index
patients
were
diagnosed
with
at
least
one
other
cancer
.
No
families
fulfilled
the
criteria
for
Cowden
syndrome
.
We
showed
a
familial
clustering
of
UC
among
relatives
of
our
index
patients
.
Of
the
seven
families
with
mutation
-verified
Lynch
Syndrome
,
only
one
had
been
previously
diagnosed
,
highlighting
the
need
to
increase
gynecologists
'
awareness
of
the
importance
of
taking
family
history
.
Our
data
on
multiple
cancers
and
young
age
of
onset
in
families
with
uterine
cancer
is
compatible
with
the
existence
of
additional
hereditary
uterine
cancer
syndromes
.