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Dedifferentiated liposarcoma with "homologous" lipoblastic (pleomorphic liposarcoma-like) differentiation: clinicopathologic and molecular analysis of a series suggesting revised diagnostic criteria.
[pleomorphic liposarcoma]
Dedifferentiated
liposarcoma
(
LPS
)
is
a
malignant
adipocytic
neoplasm
defined
as
the
transition
from
well-differentiated
LPS
to
a
nonlipogenic
sarcoma
.
Heterologous
differentiation
is
seen
in
5
%
to
10
%
of
dedifferentiated
LPS
,
usually
with
myogenic
or
osteo
/
chondrosarcomatous
elements
.
Adipocytic
differentiation
in
the
dedifferentiated
component
is
incompatible
with
the
current
definition
of
dedifferentiated
LPS
.
Pleomorphic
LPS
is
a
high
-grade
sarcoma
containing
lipoblasts
.
At
least
in
areas
,
pleomorphic
LPS
can
be
indistinguishable
from
dedifferentiated
LPS
,
except
for
the
presence
of
lipoblasts
in
pleomorphic
LPS
and
well-differentiated
LPS
areas
in
dedifferentiated
LPS
.
We
evaluated
12
unusual
liposarcomas
:
11
cases
with
pleomorphic
LPS-like
morphology
affecting
patients
with
concomitant
or
previous
well-differentiated
/
dedifferentiated
LPS
,
and
1
case
resembling
inflammatory
"
MFH
"
with
scattered
lipoblasts
.
Clinical
and
histologic
features
were
reviewed
.
Immunohistochemistry
for
MDM
2
and
CDK
4
was
carried
out
.
Amplification
of
12
q
13
to
q
15
was
studied
by
FISH
analysis
of
the
HMGA
2
locus
.
The
tumors
arose
in
the
retroperitoneum
(
7
)
,
proximal
lower
extremity
(
3
)
,
chest
wall
(
1
)
,
and
neck
(
1
)
of
9
males
and
3
females
(
median
age
66
y
;
range
49
to
76
)
.
Size
ranged
from
9
to
32
cm
(
median
23
cm
)
.
In
3
cases
,
there
was
an
abrupt
transition
between
well-differentiated
LPS
and
sheets
of
pleomorphic
lipoblasts
,
indistinguishable
from
pleomorphic
LPS
.
Four
cases
consisted
of
otherwise
typical
dedifferentiated
LPS
(
with
adjacent
well-differentiated
LPS
)
,
except
for
the
presence
of
lipoblasts
in
the
high
-grade
component
.
One
case
contained
both
nonlipogenic
spindle
cell
areas
and
an
inflammatory
"
MFH
"
-
like
component
with
numerous
admixed
lipoblasts
.
Four
cases
were
composed
exclusively
of
pleomorphic
LPS-like
areas
developing
in
1
of
the
recurrences
or
metastases
of
a
prior
typical
dedifferentiated
LPS
.
Two
cases
also
showed
heterologous
smooth
muscle
differentiation
.
MDM
2
and
CDK
4
were
positive
in
both
the
dedifferentiated
LPS
and
pleomorphic
LPS-like
components
in
12
/
12
and
11
/
12
cases
,
respectively
.
FISH
analysis
showed
high
-level
amplification
of
12
q
14
.
3
in
all
8
cases
successfully
tested
.
Karyotypes
were
available
for
3
cases
and
showed
ring
and
giant
marker
chromosomes
.
Follow-up
,
available
for
11
patients
,
ranged
from
19
to
196
months
(
median
36
mo
)
.
Seven
patients
developed
local
recurrences
(
multiple
in
3
)
,
and
3
developed
lung
metastases
.
Thus
far
,
5
patients
have
died
of
disease
,
3
are
alive
with
recurrent
or
metastatic
disease
,
and
3
are
alive
with
no
evidence
of
disease
.
We
conclude
that
dedifferentiated
LPS
can
show
lipoblastic
differentiation
in
the
high
-grade
component
,
resulting
in
areas
indistinguishable
from
pleomorphic
LPS
.
The
available
clinical
and
molecular
data
support
the
notion
of
"
homologous
"
lipoblastic
differentiation
in
dedifferentiated
LPS
,
rather
than
mixed
-
type
LPS
.
Diseases
Validation
Diseases presenting
"previous well-differentiated/dedifferentiated"
symptom
pleomorphic liposarcoma
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