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Critical histopathological analysis of 25 dedifferentiated liposarcomas, including uncommon variants, reviewed at a Tertiary Cancer Referral Center.
[pleomorphic liposarcoma]
De
-differentiated
liposarcomas
(
DDLSs
)
are
uncommon
tumors
with
a
wide
histopathological
spectrum
.
Over
an
8
-
year
period
(
2003
-
2011
)
,
25
DDLSs
,
after
review
,
were
critically
analyzed
for
histopathological
features
.
Twenty
-
five
tumors
,
in
14
men
and
11
women
(
M
:
F
=
1
.
2
:
1
)
,
with
age
range
of
22
-
88
years
(
mean
,
53
.
9
)
,
occurred
in
retroperitoneum
(
14
)
(
56
%
)
,
thigh
(
3
)
(
12
%
)
,
paratesticular
region
(
3
)
(
12
%
)
,
chest
wall
(
2
)
(
8
%
)
,
leg
(
1
)
(
4
%
)
,
shoulder
(
1
)
(
4
%
)
,
and
groin
(
1
)
(
4
%
)
.
Tumor
size
(
21
tumors
)
varied
from
7
.
5
to
25
cm
(
mean
,
17
.
5
)
.
Histopathologically
,
DD
component
was
high
grade
in
19
(
76
%
)
and
low
grade
in
6
(
24
%
)
tumors
.
Whereas
the
most
common
WD
component
was
adipocytic
type
;
the
most
common
DD
component
was
pleomorphic
sarcomatous
(
13
)
(
52
%
)
,
followed
by
myxofibrosarcomatous
(
MFS
)
-
type
(
6
)
(
24
%
)
.
Low
-grade
DD
components
included
MFS
(
2
)
,
fibrosarcoma
(
2
)
,
myogenic
/
myofibroblastic
type
(
1
)
,
and
IMFT-
type
(
1
)
.
Three
tumors
displayed
meningothelial-like
whorls
and
metaplastic
bone
formation
.
Heterologous
elements
,
noted
in
11
(
44
%
)
tumors
,
included
bone
(
8
)
and
rhabdomyoblastic
differentiation
(
2
)
.
Two
tumors
displayed
homologous
differentiation
,
reinforced
with
MDM
2
staining
.
S
100
-
P
was
diffusely
positive
in
WD
components
(
5
/
7
)
and
focally
in
DD
components
(
2
/
9
)
.
All
patients
were
treated
with
surgery
,
including
10
,
who
underwent
adjuvant
radiotherapy
.
Outcomes
(
16
patients
,
64
%
)
,
over
1
-
48
months
included
10
patients
free
of
disease
,
4
died
of
disease
,
and
2
patients
alive
with
disease
.
This
study
forms
the
largest
documentation
of
DDLSs
,
including
its
wide
histopathological
spectrum
,
from
our
country
.
Rare
cases
overlap
with
pleomorphic
liposarcoma
.
S
100
-
P
and
MDM
2
are
useful
in
substantiating
adipocytic
differentiation
,
especially
in
selected
cases
.
Analysis
of
adequate
tumor
sections
is
vital
for
correct
identification
of
a
DDLS
.
Surgical
excision
with
adjuvant
RT
forms
optimal
treatment
.
Diseases
Validation
Diseases presenting
"fibrosarcoma"
symptom
dedifferentiated liposarcoma
inclusion body myositis
liposarcoma
pleomorphic liposarcoma
well-differentiated liposarcoma
This symptom has already been validated