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Extrapulmonary lymphangioleiomyoma: clinicopathological analysis of 4 cases.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
slowly
progressive
neoplastic
disease
that
predominantly
affects
females
.
Usually
,
LAM
affects
the
lung
;
it
can
also
affect
extrapulmonary
sites
,
such
as
the
mediastinum
,
the
retroperitoneum
,
or
the
lymph
nodes
,
although
these
locations
are
rare
.
A
localized
form
of
LAM
can
manifest
as
extrapulmonary
lesions
;
this
form
is
referred
to
as
extrapulmonary
lymphangioleiomyoma
(
E
-
LAM
)
.
Due
to
the
rare
occurrence
of
E
-
LAM
and
its
variable
,
atypical
location
,
E
-
LAM
is
often
difficult
to
diagnose
.
Herein
,
we
report
the
clinicopathological
information
from
four
E
-
LAM
cases
,
and
also
review
previous
articles
investigating
this
disease
.
Four
patients
with
E
-
LAM
were
identified
at
the
Samsung
Medical
Center
(
Seoul
,
Korea
)
from
1995
to
2012
.
All
E
-
LAM
lesions
underwent
surgical
excision
.
All
patients
were
females
within
the
age
range
of
43
to
47
years
.
Two
patients
had
para-
aortic
retroperitoneal
masses
,
while
the
other
two
patients
had
pelvic
lesions
;
two
out
of
the
four
patients
also
had
accompanying
pulmonary
LAM
.
In
addition
,
no
patient
displayed
any
evidence
of
tuberous
sclerosis
.
Histologically
,
two
patients
exhibited
nuclear
atypism
with
cytologic
degeneration
.
E
-
LAM
should
be
considered
in
the
differential
diagnosis
of
patients
presenting
with
pelvic
or
para-
aortic
masses
.
We
also
conclude
that
further
clinical
and
pathological
evaluation
is
needed
in
patients
with
E
-
LAM
and
nuclear
atypism
.
Diseases
Validation
Diseases presenting
"aortic retroperitoneal masses"
symptom
lymphangioleiomyomatosis
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