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Spectrum and risk of neoplasia in Werner syndrome: a systematic review.
[werner syndrome]
Werner
syndrome
(
WS
)
is
an
autosomal
recessive
genetic
instability
and
progeroid
(
'
premature
aging
'
)
syndrome
which
is
associated
with
an
elevated
risk
of
cancer
.
Our
study
objectives
were
to
characterize
the
spectrum
of
neoplasia
in
WS
using
a
well-documented
study
population
,
and
to
estimate
the
type
-
specific
risk
of
neoplasia
in
WS
relative
to
the
general
population
.
We
obtained
case
reports
of
neoplasms
in
WS
patients
through
examining
previous
case
series
and
reviews
of
WS
,
as
well
as
through
database
searching
in
PubMed
,
Google
Scholar
,
and
J-EAST
,
a
search
engine
for
articles
from
Japan
.
We
defined
the
spectrum
(
types
and
sites
)
of
neoplasia
in
WS
using
all
case
reports
,
and
were
able
to
determine
neoplasm
type
-
specific
risk
in
Japan
WS
patients
by
calculating
standardized
incidence
and
proportionate
incidence
ratios
(
SIR
and
SPIR
,
respectively
)
relative
to
Osaka
Japan
prefecture
incidence
rates
.
We
used
a
newly
assembled
study
population
of
189
WS
patients
with
248
neoplasms
to
define
the
spectrum
of
neoplasia
in
WS
.
The
most
frequent
neoplasms
in
WS
patients
,
representing
2
/
3
of
all
reports
,
were
thyroid
neoplasms
,
malignant
melanoma
,
meningioma
,
soft
tissue
sarcomas
,
leukemia
and
pre-leukemic
conditions
of
the
bone
marrow
,
and
primary
bone
neoplasms
.
Cancer
risk
defined
by
SIRs
was
significantly
elevated
in
Japan
-resident
WS
patients
for
the
six
most
frequent
neoplasms
except
leukemia
,
ranging
from
53
.
5
-
fold
for
melanoma
of
the
skin
(
95
%
CI
:
24
.
5
,
101
.
6
)
to
8
.
9
(
95
%
CI
:
4
.
9
,
15
.
0
)
for
thyroid
neoplasms
.
Cancer
risk
as
defined
by
SPIR
was
also
significantly
elevated
for
the
most
common
malignancies
except
leukemia
.
WS
confers
a
strong
predisposition
to
several
specific
types
of
neoplasia
.
These
results
serve
as
a
guide
for
WS
clinical
care
,
and
for
additional
analyses
to
define
the
mechanistic
basis
for
cancer
in
WS
and
the
general
population
.
Diseases
Validation
Diseases presenting
"assembled study population of 189 ws patients"
symptom
werner syndrome
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