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Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases.
[pleomorphic liposarcoma]
Myxofibrosarcoma
(
MFS
)
is
one
of
the
most
common
soft
tissue
sarcomas
of
elderly
patients
and
has
a
predilection
for
the
limbs
.
Herein
,
we
report
a
previously
undescribed
variant
of
MFS
showing
epithelioid
morphology
.
Seventeen
cases
diagnosed
as
epithelioid
MFS
were
retrieved
from
the
authors
'
files
from
among
570
cases
of
MFS
.
Hematoxylin
and
eosin-stained
sections
were
reexamined
and
immunostains
for
pan-keratin
(
15
cases
)
,
S-
100
protein
(
15
)
,
desmin
(
15
)
,
and
alpha-smooth
muscle
actin
(
13
)
were
performed
.
Nine
patients
were
men
and
8
were
women
(
age
range
43
to
89
y
;
median
63
.
5
)
.
Fifteen
patients
presented
with
a
mass
,
and
in
2
of
these
there
was
also
pain
.
Duration
of
symptoms
varied
from
1
to
24
months
(
median
3
)
.
Tumor
size
ranged
from
2
to
15
cm
(
median
6
.
75
)
.
In
10
cases
,
the
tumor
was
located
in
subcutaneous
tissue
and
in
6
cases
it
was
subfascial
.
The
majority
of
the
tumors
were
located
on
the
limbs
(
8
lower
extremities
and
6
upper
extremities
)
followed
by
neck
(
1
)
,
scalp
(
1
)
,
and
trunk
(
1
)
.
Follow-up
was
available
for
14
patients
(
range
2
to
240
mo
;
median
16
)
.
Twelve
patients
were
treated
by
surgery
followed
by
chemotherapy
and
/
or
radiation
(
8
cases
)
.
One
patient
received
chemotherapy
after
an
incisional
biopsy
and
1
patient
was
treated
by
surgery
alone
.
Ten
patients
(
71
.
4
%
)
developed
local
recurrences
.
Seven
patients
(
50
%
)
developed
metastases
to
lungs
or
retroperitoneum
.
Five
patients
(
35
.
7
%
)
have
died
of
disease
so
far
.
Two
patients
were
lost
to
follow-up
.
Morphologically
,
14
cases
were
high
grade
,
2
were
intermediate
,
and
1
was
low
grade
.
Tumors
were
characterized
by
a
multinodular
,
infiltrating
growth
pattern
with
alternation
of
hypercellular
and
hypocellular
myxoid
areas
;
the
latter
showed
prominent
curvilinear
vessels
.
Neoplastic
cells
were
arranged
singly
and
in
small
clusters
in
the
myxoid
areas
or
formed
sheets
in
the
hypercellular
areas
,
where
they
showed
epithelioid
morphology
with
round
nuclei
,
vesicular
chromatin
,
prominent
nucleoli
,
and
moderate
amounts
of
eosinophilic
cytoplasm
.
The
epithelioid
areas
were
generally
multifocal
with
admixed
areas
of
conventional
MFS
.
Immunostains
were
negative
for
all
markers
studied
.
Differential
diagnosis
included
carcinoma
,
melanoma
,
myoepithelial
carcinoma
,
pleomorphic
liposarcoma
,
and
pleomorphic
rhabdomyosarcoma
.
In
conclusion
,
epithelioid
MFS
is
a
rare
variant
of
MFS
,
accounting
for
<
3
%
of
MFS
in
consultation
material
.
Its
natural
history
seems
more
aggressive
than
usual
high
-grade
MFS
,
with
approximately
70
%
local
recurrence
and
50
%
metastases
,
even
within
a
relatively
short
follow-up
period
.
Diseases
Validation
Diseases presenting
"round nuclei"
symptom
erdheim-chester disease
pleomorphic liposarcoma
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