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[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
.
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