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Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features.
[pleomorphic liposarcoma]
Liposarcoma
rarely
occurs
in
the
mediastinum
,
and
most
reports
predate
the
current
genetically
based
classification
system
.
We
report
the
clinicopathologic
and
molecular
genetic
features
of
a
series
of
thoracic
liposarcomas
identified
over
a
60
-
year
period
.
Twenty
-
four
confirmed
cases
were
reclassified
using
the
most
recent
World
Health
Organization
classification
.
Fluorescent
in
situ
hybridization
for
CPM
amplification
and
/
or
DDIT
3
rearrangement
was
performed
on
selected
cases
.
The
24
cases
occurred
in
13
men
and
11
women
(
mean
age
,
53
y
;
range
,
15
to
73
y
)
and
arose
in
all
mediastinal
compartments
.
All
subtypes
were
encountered
with
8
well-differentiated
liposarcomas
,
6
dedifferentiated
liposarcomas
(
3
of
6
confirmed
CPM
+
)
,
7
pleomorphic
liposarcomas
(
2
of
7
confirmed
CPM
-
,
1
of
7
confirmed
DDIT
3
-
)
,
2
myxoid
liposarcomas
,
and
1
unclassifiable
liposarcoma
(
CPM
-
and
DDIT
3
-
)
.
Unusual
histologic
features
included
myxoid
well-differentiated
liposarcoma
mimicking
myxoid
liposarcoma
(
2
cases
)
,
lipoleiomyosarcoma
(
1
case
)
,
dedifferentiated
liposarcoma
with
"
meningothelial
"
-
like
dedifferentiation
,
differentiated
myxoid
liposarcoma
mimicking
well-differentiated
liposarcoma
(
CPM
-
)
,
and
pleomorphic
liposarcoma
with
epithelioid
and
myxoid
change
.
Follow-up
information
was
available
for
19
patients
(
mean
,
55
mo
;
range
,
8
to
252
mo
)
.
Outcome
was
strongly
associated
with
histologic
subtype
,
with
death
from
disease
occurring
in
1
of
6
well-differentiated
,
1
of
4
dedifferentiated
,
5
of
7
pleomorphic
,
and
2
of
2
myxoid
liposarcomas
.
The
mediastinum
shows
a
preponderance
of
uncommon
subtypes
and
unusual
morphologic
variants
.
Correct
classification
has
important
implications
,
with
most
patients
with
well-differentiated
/
dedifferentiated
liposarcoma
having
a
protracted
clinical
course
,
in
contrast
to
the
more
rapid
disease
progression
seen
in
patients
with
myxoid
and
pleomorphic
liposarcoma
.
Diseases
Validation
Diseases presenting
"unusual morphologic variants"
symptom
dedifferentiated liposarcoma
pleomorphic liposarcoma
well-differentiated liposarcoma
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