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Are peripheral purely undifferentiated pleomorphic sarcomas with MDM2 amplification dedifferentiated liposarcomas?
[well-differentiated liposarcoma]
Dedifferentiated
liposarcoma
(
DDLPS
)
has
been
defined
as
a
tumor
composed
of
well-differentiated
liposarcoma
associated
with
a
nonlipogenic
undifferentiated
sarcoma
and
is
genetically
characterized
by
a
12
q
13
-
15
amplicon
with
MDM
2
amplification
.
Some
peripheral
(
extremities
,
trunk
wall
,
head
/
neck
)
undifferentiated
pleomorphic
sarcomas
(
UPS
)
without
areas
of
well-differentiated
liposarcoma
present
an
MDM
2
amplification
.
We
addressed
whether
they
are
true
DDLPS
or
not
.
We
compared
the
clinical
data
,
histologic
data
,
MDM
2
status
(
immunohistochemistry
[
IHC
]
,
fluorescence
in
situ
hybridization
[
FISH
]
)
,
genomic
profile
(
array
comparative
genomic
hybridization
)
,
and
follow-up
of
19
patients
with
peripheral
UPS
with
MDM
2
amplification
and
62
with
peripheral
conventional
DDLPS
retrieved
from
the
French
sarcoma
network
(
RRePS
)
and
the
Conticabase
(
Connective
Tissue
Cancer
Network
database
)
.
For
a
control
cohort
,
we
described
153
patients
from
the
Conticabase
,
with
peripheral
UPS
without
expression
of
MDM
2
by
IHC
.
By
IHC
,
tumor
cells
were
positive
for
MDM
2
in
59
conventional
DDLPS
and
in
all
UPS
with
MDM
2
amplification
.
FISH
analysis
and
/
or
quantitative
polymerase
chain
reaction
showed
amplification
of
MDM
2
in
54
conventional
DDLPS
and
in
all
UPS
with
MDM
2
amplification
.
The
2
-
year
overall
survival
rates
of
UPS
with
MDM
2
amplification
,
conventional
DDLPS
,
and
UPS
without
expression
of
MDM
2
were
93
.
3
%
,
90
.
7
%
,
and
73
.
9
%
,
respectively
.
Such
similarities
in
the
clinical
characteristics
,
morphology
,
genomic
profile
,
and
follow-up
of
peripheral
UPS
with
MDM
2
amplification
and
peripheral
conventional
DDLPS
strongly
suggest
that
peripheral
UPS
with
MDM
2
amplification
are
in
fact
DDLPS
.
Faced
with
histologic
diagnosis
of
UPS
,
a
systematic
IHC
evaluation
of
MDM
2
allows
a
selection
of
cases
for
FISH
analysis
permitting
the
diagnosis
of
DDLPS
.
Diseases
Validation
Diseases presenting
"a nonlipogenic undifferentiated sarcoma"
symptom
dedifferentiated liposarcoma
well-differentiated liposarcoma
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