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Intraperitoneal dedifferentiated liposarcoma showing MDM2 amplification: case report.
[well-differentiated 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
"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