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Atypical lipomatous tumor, its variants, and its combined forms: a study of 61 cases, with a minimum follow-up of 10 years.
[pleomorphic liposarcoma]
Sixty
-
one
cases
of
neoplasms
composed
wholly
or
in
part
of
atypical
lipomatous
tumor
were
reviewed
.
Minimum
follow-up
was
10
years
.
The
cases
were
divided
into
4
groups
based
on
the
findings
in
the
initial
excision
specimen
:
conventional
atypical
lipomatous
tumor
(
n
=
15
)
,
cellular
atypical
lipomatous
tumor
(
n
=
21
)
,
dedifferentiated
liposarcoma
(
n
=
24
)
,
and
atypical
lipomatous
tumor
with
a
pleomorphic
liposarcoma
-like
component
(
n
=
1
)
.
The
term
"
cellular
atypical
lipomatous
tumor
"
was
applied
to
atypical
lipomatous
tumors
having
areas
of
increased
cellularity
that
when
non-lipogenic
lacked
the
5
mitotic
figures
per
10
high
-power
fields
(
maximal
rate
)
required
for
a
dedifferentiated
component
and
when
lipogenic
fell
short
of
being
truly
pleomorphic
liposarcoma
-like
.
Myxoid
regions
within
this
spectrum
sometimes
had
prominent
or
even
plexiform
vascularity
,
creating
a
resemblance
to
myxoid
liposarcoma
especially
when
interspersed
small
fat
cells
were
present
.
The
most
important
prognostic
factor
was
tumor
location
,
as
none
of
the
12
patients
with
a
subcutaneous
or
intramuscular
neoplasm
died
of
tumor
.
Among
the
49
patients
with
neoplasms
of
central
body
sites
(
mostly
retroperitoneum
)
,
those
with
dedifferentiated
liposarcoma
had
significantly
shorter
survival
(
median
77
mo
)
than
those
with
cellular
(
median
142
mo
)
or
conventional
(
median
209
mo
)
atypical
lipomatous
tumor
,
whereas
there
was
no
statistically
significant
difference
between
the
latter
2
categories
.
Patients
with
atypical
lipomatous
tumor
(
either
cellular
or
conventional
)
in
central
body
sites
had
significantly
shorter
survival
if
the
tumor
transformed
into
dedifferentiated
liposarcoma
in
recurrence
,
and
,
conversely
,
those
with
central
body
site
dedifferentiated
liposarcoma
had
significantly
longer
survival
if
it
recurred
as
atypical
lipomatous
tumor
.
Metastasis
(
7
cases
)
occurred
only
when
the
initial
specimen
or
a
recurrence
demonstrated
dedifferentiated
liposarcoma
.
Diseases
Validation
Diseases presenting
"and"
symptom
achondroplasia
adrenomyeloneuropathy
aniridia
carcinoma of the gallbladder
cutaneous mastocytosis
cystinuria
esophageal squamous cell carcinoma
harlequin ichthyosis
hodgkin lymphoma, classical
hydrocephalus with stenosis of the aqueduct of sylvius
kallmann syndrome
liposarcoma
locked-in syndrome
neonatal adrenoleukodystrophy
omenn syndrome
oral submucous fibrosis
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
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