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Dedifferentiated liposarcoma with "homologous" lipoblastic (pleomorphic liposarcoma-like) differentiation: clinicopathologic and molecular analysis of a series suggesting revised diagnostic criteria.
[pleomorphic liposarcoma]
Dedifferentiated
liposarcoma
(
LPS
)
is
a
malignant
adipocytic
neoplasm
defined
as
the
transition
from
well-differentiated
LPS
to
a
nonlipogenic
sarcoma
.
Heterologous
differentiation
is
seen
in
5
%
to
10
%
of
dedifferentiated
LPS
,
usually
with
myogenic
or
osteo
/
chondrosarcomatous
elements
.
Adipocytic
differentiation
in
the
dedifferentiated
component
is
incompatible
with
the
current
definition
of
dedifferentiated
LPS
.
Pleomorphic
LPS
is
a
high
-grade
sarcoma
containing
lipoblasts
.
At
least
in
areas
,
pleomorphic
LPS
can
be
indistinguishable
from
dedifferentiated
LPS
,
except
for
the
presence
of
lipoblasts
in
pleomorphic
LPS
and
well-differentiated
LPS
areas
in
dedifferentiated
LPS
.
We
evaluated
12
unusual
liposarcomas
:
11
cases
with
pleomorphic
LPS-like
morphology
affecting
patients
with
concomitant
or
previous
well-differentiated
/
dedifferentiated
LPS
,
and
1
case
resembling
inflammatory
"
MFH
"
with
scattered
lipoblasts
.
Clinical
and
histologic
features
were
reviewed
.
Immunohistochemistry
for
MDM
2
and
CDK
4
was
carried
out
.
Amplification
of
12
q
13
to
q
15
was
studied
by
FISH
analysis
of
the
HMGA
2
locus
.
The
tumors
arose
in
the
retroperitoneum
(
7
)
,
proximal
lower
extremity
(
3
)
,
chest
wall
(
1
)
,
and
neck
(
1
)
of
9
males
and
3
females
(
median
age
66
y
;
range
49
to
76
)
.
Size
ranged
from
9
to
32
cm
(
median
23
cm
)
.
In
3
cases
,
there
was
an
abrupt
transition
between
well-differentiated
LPS
and
sheets
of
pleomorphic
lipoblasts
,
indistinguishable
from
pleomorphic
LPS
.
Four
cases
consisted
of
otherwise
typical
dedifferentiated
LPS
(
with
adjacent
well-differentiated
LPS
)
,
except
for
the
presence
of
lipoblasts
in
the
high
-grade
component
.
One
case
contained
both
nonlipogenic
spindle
cell
areas
and
an
inflammatory
"
MFH
"
-
like
component
with
numerous
admixed
lipoblasts
.
Four
cases
were
composed
exclusively
of
pleomorphic
LPS-like
areas
developing
in
1
of
the
recurrences
or
metastases
of
a
prior
typical
dedifferentiated
LPS
.
Two
cases
also
showed
heterologous
smooth
muscle
differentiation
.
MDM
2
and
CDK
4
were
positive
in
both
the
dedifferentiated
LPS
and
pleomorphic
LPS-like
components
in
12
/
12
and
11
/
12
cases
,
respectively
.
FISH
analysis
showed
high
-level
amplification
of
12
q
14
.
3
in
all
8
cases
successfully
tested
.
Karyotypes
were
available
for
3
cases
and
showed
ring
and
giant
marker
chromosomes
.
Follow-up
,
available
for
11
patients
,
ranged
from
19
to
196
months
(
median
36
mo
)
.
Seven
patients
developed
local
recurrences
(
multiple
in
3
)
,
and
3
developed
lung
metastases
.
Thus
far
,
5
patients
have
died
of
disease
,
3
are
alive
with
recurrent
or
metastatic
disease
,
and
3
are
alive
with
no
evidence
of
disease
.
We
conclude
that
dedifferentiated
LPS
can
show
lipoblastic
differentiation
in
the
high
-grade
component
,
resulting
in
areas
indistinguishable
from
pleomorphic
LPS
.
The
available
clinical
and
molecular
data
support
the
notion
of
"
homologous
"
lipoblastic
differentiation
in
dedifferentiated
LPS
,
rather
than
mixed
-
type
LPS
.
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