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Recurrent retroperitoneal liposarcoma in a patient with neurofibromatosis type I.
[dedifferentiated liposarcoma]
Liposarcoma
complicating
neurofibromatosis
is
very
rare
.
Only
a
few
cases
have
been
described
until
now
.
We
present
a
case
of
recurrent
dedifferentiated
retroperitoneal
liposarcoma
in
a
patient
with
neurofibromatosis
type
I
(
NF-
1
)
.
A
47
-
year
-old
Caucasian
woman
with
NF-
1
presented
to
the
hospital
initially
complaining
of
left
lumbar
pain
irradiating
to
the
anterior
thigh
and
knee
.
Physical
examination
showed
atrophy
of
the
lower
extremities
bilaterally
and
decreased
motor
strength
on
the
left
lower
extremity
.
Radiological
studies
demonstrated
an
enhancing
lesion
in
the
left
paraspinal
region
,
suggesting
malignancy
.
The
patient
underwent
local
resection
of
tumour
with
safety
margins
.
Pathological
examination
was
consistent
with
dedifferentiated
liposarcoma
(
DDLS
)
with
positivity
for
MDM
2
and
CDK
4
markers
.
No
evidence
of
metastasis
was
noted
on
the
radiological
studies
.
The
final
diagnosis
was
DDLS
,
high
-grade
(
G
3
)
,
pT
2
bN
0
M
0
,
stage
III
.
After
6
weeks
post-tumour
resection
,
the
patient
experienced
recurrence
of
malignancy
.
Chemotherapy
with
cisplatin
and
doxorubicin
was
initiated
in
the
patient
.
Liposarcoma
in
the
context
of
neurofibromatosis
is
very
rare
.
To
the
best
of
our
knowledge
,
only
six
cases
have
been
reported
until
now
in
the
literature
.
We
are
presenting
this
case
to
underline
the
possibility
of
recurrence
in
the
case
of
retroperitoneal
DDLSs
despite
local
tumour
resection
.
Also
,
although
the
role
of
chemotherapy
is
controversial
we
decided
to
start
treatment
with
cisplatin
and
doxorubicin
given
the
success
of
chemotherapy
in
similar
case
presentations
.
