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Dedifferentiated liposarcoma with homologous lipoblastic differentiation: expanding the spectrum to include low-grade tumours.
[well-differentiated liposarcoma]
Dedifferentiated
liposarcoma
(
DDLPS
)
is
traditionally
defined
as
a
non-lipogenic
high
-grade
sarcoma
arising
from
a
well-differentiated
liposarcoma
that
confers
metastatic
potential
.
Recently
,
DDLPSs
with
lipoblastic
differentiation
,
i
.
e
.
morphologically
lipogenic
DDLPSs
,
were
reported
.
Because
of
the
lipoblastic
differentiation
,
these
tumours
caused
confusion
,
and
were
reported
under
different
names
.
However
,
cytogenetic
and
molecular
studies
have
revealed
their
DDLPS
nature
.
So
far
,
the
cases
reported
have
been
high
-grade
pleomorphic
liposarcoma
-like
tumours
.
In
this
study
we
have
collected
another
series
that
contains
low
-grade
tumours
,
and
expand
the
histological
spectrum
.
Eighteen
cases
of
DDLPS
with
lipoblastic
differentiation
from
various
anatomical
locations
were
analysed
by
routine
histology
,
immunohistochemistry
,
and
MDM
2
fluorescence
in
-situ
hybridization
.
Two
main
histological
patterns
were
seen
:
one
featured
a
spindle
cell
sarcoma
containing
lipoblasts
with
variable
nuclear
pleomorphism
,
and
the
other
a
pleomorphic
liposarcoma
-like
tumour
including
the
epithelioid
variant
.
Two
cases
showed
low
nuclear
grade
and
lipogenic
activity
in
the
metastatic
foci
.
CDK
4
,
MDM
2
and
p
16
(
INK
)
(
4
a
)
overexpression
was
seen
in
all
except
one
case
.
MDM
2
amplification
was
found
in
all
16
cases
tested
.
We
have
expanded
the
spectrum
of
this
variant
of
DDLPS
to
include
low
-grade
tumours
,
in
which
a
careful
search
for
increased
mitotic
activity
is
essential
.
Like
conventional
DDLPS
,
these
tumours
are
capable
of
metastasis
.
Diseases
Validation
Diseases presenting
"in which a careful search for increased mitotic activity is essential"
symptom
dedifferentiated liposarcoma
pleomorphic liposarcoma
well-differentiated liposarcoma
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