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Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution.
[pleomorphic liposarcoma]
The
objective
of
this
study
was
to
investigate
prognostic
factors
and
clinical
outcome
of
myxoid
/
round
cell
and
pleomorphic
liposarcoma
.
Three
hundred
twenty-
nine
patients
with
localized
myxoid
/
round
cell
or
pleomorphic
liposarcoma
who
underwent
surgery
at
the
Istituto
Nazionale
per
lo
Studio
e
la
Cura
dei
Tumori
(
Milan
,
Italy
)
over
25
years
were
reviewed
.
The
rates
of
local
recurrence
,
distant
metastases
,
and
survival
were
studied
.
Two
hundred
fourteen
patients
presented
with
primary
disease
,
and
115
patients
had
locally
recurrent
tumors
.
The
disease-
specific
survival
rate
was
75
%
at
10
years
,
and
the
local
recurrence
and
distant
metastases
incidence
were
25
%
and
15
%
,
respectively
.
Presentation
with
recurrent
disease
,
tumor
size
(
>
10
cm
)
,
tumor
grade
(
French
Federation
of
Cancer
Centers
grade
II
or
III
vs
grade
I
)
,
and
positive
surgical
margins
were
independent
predictors
of
death
.
Tumor
site
and
radiation
therapy
also
played
a
role
,
mostly
related
to
their
effect
on
local
outcome
.
Pathologic
grade
and
histologic
subtype
influenced
distant
metastases
.
Extrapulmonary
metastases
were
associated
with
poorer
postmetastatic
disease-
specific
survival
.
Myxoid
/
round
cell
liposarcomas
shared
similar
prognostic
factors
with
other
soft
tissue
sarcomas
and
had
a
relatively
good
clinical
outcome
.
The
presence
of
>
5
%
of
round
cell
component
singled
out
a
group
of
patients
at
greater
risk
of
metastases
and
death
but
with
a
broad
spectrum
of
disease
aggressiveness
.
Extrapulmonary
metastases
were
a
peculiar
pattern
of
myxoid
/
round
cell
liposarcoma
that
require
special
consideration
for
treatment
and
prognosis
.
Diseases
Validation
Diseases presenting
"cancer"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
cadasil
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated