Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
[A case of incidentally diagnosed retroperitoneal lymphangioleiomyomatosis with no respiratory symptoms].
[lymphangioleiomyomatosis]
A
39
-
year
-old
woman
presented
with
a
large
retroperitoneal
tumor
found
incidentally
in
a
routine
examination
.
The
138
×
37
×
26
mm
mass
was
located
in
the
left
paraaortic
region
.
Blood
tests
and
urinalyses
including
endocrinological
examinations
revealed
no
abnormalities
.
A
chest
computed
tomography
revealed
multiple
thin
-walled
pulmonary
cysts
,
which
is
a
characteristic
of
lymphangioleiomyomatosis
(
LAM
)
.
Because
the
findings
strongly
suggested
that
the
retroperitoneal
tumor
was
an
extrapulmonary
manifestion
of
LAM
,
we
performed
laparoscopic
resection
of
the
tumor
for
diagnosis
and
treatment
.
The
pathological
diagnosis
was
LAM
.
The
tumor
cells
were
immunohistochemically
positive
for
α
-
smooth
muscle
actin
and
weakly
positive
for
HMB
45
,
which
is
consistent
with
LAM
.
The
cells
were
also
positive
for
estrogen
receptor
(
ER
)
and
progesterone
receptor
(
PgR
)
.
LAM
is
a
rare
progressive
disease
that
affects
mainly
the
lung
,
and
leads
to
chronic
respiratory
failure
.
Extrapulmonary
LAM
without
respiratory
symptoms
,
is
extremely
rare
.
In
the
past
,
the
prognosis
of
LAM
was
poor
,
with
a
median
survival
of
8
-
10
years
,
but
now
85
%
survive
more
than
10
years
.
In
the
present
case
,
deterioration
of
pulmonary
lesions
was
not
observed
during
the
10
months
follow-up
.
Because
ERand
PgRfindings
were
positive
,
we
will
consider
hormonal
therapy
as
a
treatment
option
,
when
the
pulmonary
lesions
progress
in
the
present
case
.
Diseases
Validation
Diseases presenting
"respiratory symptoms"
symptom
allergic bronchopulmonary aspergillosis
esophageal carcinoma
legionellosis
lymphangioleiomyomatosis
scrub typhus
typhoid
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom