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Liposarcoma arising in uterine lipoleiomyoma: a report of 3 cases and review of the literature.
[pleomorphic liposarcoma]
Primary
sarcomas
of
the
uterus
are
uncommon
,
leiomyosarcoma
being
the
most
frequent
.
Most
uterine
sarcomas
arise
de
novo
,
with
malignant
transformation
of
a
benign
mesenchymal
tumor
being
a
very
rare
event
,
and
is
reported
only
in
leiomyomata
.
The
clinicopathologic
features
of
3
uterine
liposarcomas
arising
in
association
with
a
lipoleiomyoma
were
studied
.
Immunohistochemistry
for
desmin
,
h-caldesmon
,
S
100
,
and
MDM
2
,
and
fluorescence
in
situ
hybridization
for
the
t
(
12
;
16
)
(
q
13
;
p
11
)
were
performed
in
all
cases
.
Patients
ranged
in
age
from
49
to
70
(
mean
,
59
)
years
.
The
tumors
were
centered
in
the
myometrium
,
ranged
in
size
from
10
to
18
.
5
cm
,
and
showed
a
gelatinous
cut
surface
with
foci
of
necrosis
.
On
microscopic
examination
,
the
tumors
had
well-circumscribed
pushing
margins
.
One
neoplasm
was
uniformly
hypocellular
with
a
prominent
myxoid
background
,
and
a
striking
delicate
vascular
network
.
Another
neoplasm
showed
alternating
hypocellular
(
myxoid
)
and
hypercellular
areas
,
whereas
the
third
tumor
was
uniformly
hypercellular
with
a
hyalinized
background
.
In
the
myxoid
areas
,
the
cells
were
small
and
spindle
with
oval
nuclei
and
inconspicuous
nucleoli
.
In
the
hypercellular
areas
,
the
cells
were
pleomorphic
with
large
,
hyperchromatic
nuclei
.
Mitotic
activity
ranged
from
<
3
to
7
/
10
high
-power
fields
.
Lipoblasts
were
present
in
all
tumors
but
were
more
common
in
the
hypercellular
areas
.
Two
tumors
merged
imperceptibly
with
a
lipoleiomyoma
(
1
typical
and
1
with
bizarre
nuclei
)
,
whereas
the
third
tumor
showed
an
infarcted
area
composed
of
ghost
mature
adipocytes
admixed
with
hyalinized
smooth
muscle
most
consistent
with
an
infarcted
lipoleiomyoma
.
Tumors
were
classified
as
myxoid
,
mixed
myxoid
and
pleomorphic
,
and
pleomorphic
liposarcoma
,
respectively
.
The
benign
and
malignant
adipose
components
were
positive
for
S
100
,
whereas
the
benign
smooth
muscle
component
stained
for
desmin
and
h-caldesmon
.
MDM
2
immunostain
was
positive
in
the
2
cases
with
a
pleomorphic
liposarcoma
component
.
Fluorescence
in
situ
hybridization
analysis
was
successfully
completed
in
only
1
of
3
tumors
(
pure
pleomorphic
liposarcoma
)
,
which
failed
to
show
the
t
(
12
;
16
)
and
HMAG
2
amplification
.
The
patients
are
alive
and
well
1
,
2
,
and
20
years
after
initial
surgery
with
no
adjuvant
therapy
.
Primary
liposarcomas
of
the
uterus
are
extremely
rare
and
are
most
likely
to
arise
from
malignant
transformation
of
a
lipoleiomyoma
.
These
tumors
should
be
added
to
the
differential
diagnosis
of
benign
lipomatous
tumors
,
myxoid
mesenchymal
tumors
,
and
malignant
mixed
Müllerian
tumors
(
if
pleomorphic
)
of
the
uterus
.
Diseases
Validation
Diseases presenting
"neoplasm"
symptom
adrenal incidentaloma
alexander disease
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
erdheim-chester disease
esophageal adenocarcinoma
esophageal carcinoma
familial hypocalciuric hypercalcemia
focal myositis
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
This symptom has already been validated
