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Pleomorphic liposarcoma of the uterine corpus with focal smooth muscle differentiation.
[pleomorphic liposarcoma]
We
describe
a
rare
case
of
a
pleomorphic
liposarcoma
(
>
95
%
)
of
the
uterine
corpus
with
focal
leiomyosarcomatous
(
<
5
%
)
differentiation
,
and
review
the
literature
on
10
purely
mesenchymal
liposarcomatous
tumors
of
the
uterine
corpus
reported
earlier
.
The
patients
,
including
this
case
,
ranged
in
age
from
45
to
78
years
(
mean
and
median
59
y
)
and
frequently
presented
with
abnormal
uterine
bleeding
and
/
or
abdominal
pain
.
The
tumors
were
typically
large
(
average
tumor
size
15
.
79
cm
,
range
:
7
to
28
cm
)
,
and
displayed
a
cut
surface
with
hemorrhage
and
gross
degeneration
.
Most
tumors
were
confined
to
the
uterus
after
initial
evaluation
.
Follow
up
was
available
in
10
(
91
%
)
of
the
11
patients
.
Five
(
50
%
)
of
the
10
patients
showed
no
evidence
of
tumor
recurrence
or
metastases
at
an
average
follow-up
of
30
.
4
months
.
The
remaining
5
patients
experienced
recurrence
within
the
first
year
of
their
extirpative
operations
.
However
,
2
(
40
%
)
of
the
5
patients
who
showed
no
recurrence
had
limited
follow-up
(
2
and
6
mo
)
.
After
excluding
cases
without
follow-up
and
nonrecurring
cases
with
less
than
1
-
year
follow-up
,
the
overall
rate
of
recurrence
or
metastases
was
62
.
5
%
(
5
of
8
)
.
Eight
(
72
.
7
%
)
of
the
11
reported
cases
were
associated
with
smooth
muscle
neoplasia
,
including
2
cases
designated
as
"
mesenchymoma
"
with
liposarcomatous
,
leiomyosarcomatous
,
and
osteosarcomatous
elements
;
1
case
of
a
myxoid
liposarcoma
arising
from
a
leiomyoma
;
and
5
cases
of
tumors
with
varying
amounts
of
leiomyosarcomatous
and
liposarcomatous
components
.
On
the
basis
of
this
review
,
we
postulate
that
a
significant
subset
of
liposarcomatous
tumors
of
the
uterus
may
actually
represent
an
unusual
form
of
divergent
differentiation
of
leiomyosarcomas
.
This
may
have
adjuvant
management
implications
for
patients
with
these
rare
tumors
.
Diseases
Validation
Diseases presenting
"abdominal pain"
symptom
22q11.2 deletion syndrome
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
child syndrome
cholangiocarcinoma
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
erdheim-chester disease
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
focal myositis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
typhoid
von hippel-lindau disease
well-differentiated liposarcoma
This symptom has already been validated