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Primary liposarcoma of the mediastinum.
[pleomorphic liposarcoma]
Patient
.
A
62
-
year
-old
man
presented
with
effort
dyspnea
,
non-productive
cough
and
weakness
of
4
month
duration
.
He
had
no
findings
on
physical
examination
.
Discussion
.
Chest
X-
ray
revealed
a
large
mass
in
the
left
anterior
mediastinum
.
Computerized
tomography
of
the
chest
showed
a
well-delineated
homogeneous
mediastinal
mass
with
fat-equivalent
density
and
a
small
pleural
effusion
.
Fiberoptic
bronchoscopy
revealed
narrowing
of
the
left
main
bronchus
,
secondary
to
external
compression
.
The
bronchial
mucosa
was
normal
and
brush
cytology
was
negative
.
A
CT
-guided
fine
needle
aspiration
(
FNA
)
of
the
mass
yielded
fragments
of
cells
embedded
in
myxoid
background
material
and
closely
packed
atypical
lipoblasts
,
compatible
with
liposarcoma
.
The
patient
underwent
a
left
lateral
thoracotomy
and
margibnal
resection
of
the
mass
.
The
histopathological
examination
confirmed
the
diagnosis
of
mixed
-
type
liposarcoma
,
consisted
of
myxoid
and
pleomorphic
liposarcoma
.
Postoperative
two
-field
radiation
therapy
was
delivered
to
the
mediastinum
for
a
total
midplane
dose
of
40
Gy
.
After
a
disease-free
interval
of
8
months
the
disease
recurred
in
the
mediastinum
and
pleura
.
Palliative
chemotherapy
achieved
a
short
duration
partial
response
but
the
patient
succumbed
to
local
recurrence
2
years
after
the
diagnosis
.
Diseases
Validation
Diseases presenting
"partial response"
symptom
cholangiocarcinoma
dedifferentiated liposarcoma
esophageal squamous cell carcinoma
hirschsprung disease
hodgkin lymphoma, classical
junctional epidermolysis bullosa
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary hyperoxaluria type 1
severe combined immunodeficiency
waldenström macroglobulinemia
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