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Intraperitoneal dedifferentiated liposarcoma showing MDM2 amplification: case report.
[dedifferentiated liposarcoma]
Liposarcoma
is
the
most
common
type
of
soft
tissue
sarcoma
(
STS
)
.
It
is
divided
into
five
groups
according
to
histological
pattern
:
well-differentiated
,
myxoid
,
round
cell
,
pleomorphic
,
and
dedifferentiated
.
Dedifferentiated
liposarcoma
most
commonly
occurs
in
the
retroperitoneum
,
while
an
intraperitoneal
location
is
extremely
rare
.
Only
seven
cases
have
been
reported
in
literature
.
Many
pathologists
recognize
that
a
large
number
of
intra-
abdominal
poorly
differentiated
sarcomas
are
dedifferentiated
liposarcomas
.
We
report
a
case
initially
diagnosed
as
undifferentiated
sarcoma
that
was
reclassified
as
intraperitoneal
dedifferentiated
liposarcoma
showing
an
amplification
of
the
MDM
2
gene
.
A
59
-
year
-old
woman
with
abdominal
pain
and
constipation
was
referred
to
the
Department
of
Advanced
Biomedical
Sciences
,
University
of
Naples
Federico
II
,
Naples
,
Italy
,
in
November
2012
.
On
physical
examination
,
a
very
large
firm
mass
was
palpable
in
the
meso-hypogastrium
.
Computed
tomography
(
CT
)
scan
showed
a
heterogeneous
density
mass
(
measuring
10
×
19
cm
)
that
was
contiguous
with
the
mesentery
and
compressed
the
third
part
of
the
duodenum
and
jejunum
.
At
laparotomy
,
a
large
mass
occupying
the
entire
abdomen
was
found
,
adhering
to
the
first
jejunal
loop
and
involving
the
mesentery
.
Surgical
removal
of
the
tumor
along
with
a
jejunal
resection
was
performed
because
the
first
jejunal
loop
was
firmly
attached
to
the
tumor
.
Macroscopic
examination
showed
a
solid
,
whitish
,
cerebroid
,
and
myxoid
mass
,
with
variable
hemorrhage
and
cystic
degeneration
,
measuring
26
×
19
×
5
cm
.
Microscopic
examination
revealed
two
main
different
morphologic
patterns
:
areas
with
spindle
cells
in
a
myxoid
matrix
and
areas
with
pleomorphic
cells
.
The
case
was
initially
diagnosed
as
undifferentiated
pleomorphic
sarcoma
.
Histological
review
showed
areas
of
well-differentiated
liposarcoma
.
Fluorescence
in
situ
hybridization
(
FISH
)
analysis
was
performed
and
demonstrated
an
amplification
of
the
MDM
2
gene
.
Definitive
diagnosis
was
intraperitoneal
dedifferentiated
liposarcoma
.
No
adjuvant
therapy
was
given
,
but
5
months
after
laparotomy
,
the
patient
presented
with
a
locoregional
recurrence
and
chemotherapy
with
high
-dose
ifosfamide
was
started
.
No
guidelines
are
available
for
the
management
of
intraperitoneal
dedifferentiated
liposarcoma
.
We
report
this
case
to
permit
the
collection
of
a
larger
number
of
cases
to
improve
understanding
and
management
of
this
tumor
.
Moreover
,
this
study
strongly
suggests
that
poorly
differentiated
sarcomas
should
prompt
extensive
sampling
to
demonstrate
a
well-differentiated
liposarcoma
component
and
,
if
possible
,
FISH
analysis
.
Diseases
Validation
Diseases presenting
"large number"
symptom
acute rheumatic fever
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
canavan disease
coats disease
cowden syndrome
dedifferentiated liposarcoma
dracunculiasis
epidermolysis bullosa simplex
fabry disease
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
kindler syndrome
legionellosis
malignant atrophic papulosis
neuralgic amyotrophy
phenylketonuria
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
severe combined immunodeficiency
triple a syndrome
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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