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In pulmonary lymphangioleiomyomatosis expression of progesterone receptor is frequently higher than that of estrogen receptor.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
of
the
lung
is
a
rare
low
-grade
malignancy
affecting
primarily
women
of
childbearing
age
.
LAM
is
characterized
by
the
proliferation
of
SMA
and
HMB-
45
positive
spindle-shaped
and
epithelioid
cells
throughout
the
lung
in
the
form
of
discrete
lesions
causing
cystic
destruction
and
ultimately
respiratory
insufficiency
.
LAM
occurs
sporadically
or
in
patients
with
tuberous
sclerosis
complex
(
TSC
)
and
is
etiologically
linked
to
mutations
in
the
TSC
1
and
TSC
2
genes
.
Although
LAM
cells
are
known
to
express
estrogen
and
progesterone
receptors
(
ER
and
PR
,
respectively
)
,
their
respective
expression
level
was
never
determined
.
Therefore
,
here
we
measured
the
immunohistochemical
expression
of
ERs
and
PRs
in
a
large
series
of
pulmonary
LAM
cases
using
the
Aperio
Spectrum
Analysis
Platform
.
Our
case
series
comprised
open
lung
biopsy
specimens
from
20
LAM
patients
and
lungs
explanted
during
the
course
of
lung
transplant
from
24
patients
.
All
cases
were
positive
for
ER
and
PR
.
PR
expression
was
statistically
significantly
higher
than
ER
in
80
%
of
the
biopsies
while
ER
predominated
only
in
one
case
.
Specimens
from
explanted
cases
of
LAM
had
relatively
fewer
PR-
positive
nuclei
.
As
a
result
,
PR
expression
was
significantly
higher
than
ER
in
38
%
of
the
cases
,
whereas
ER
predominated
in
33
%
.
Overall
,
PR
expression
predominated
in
57
%
of
cases
and
ER
in
21
%
.
These
data
indicate
that
PR
frequently
prevails
over
ER
in
pulmonary
LAM
.
LAM
is
unusual
in
its
high
PR
/
ER
ratio
;
other
female
neoplasms
show
a
definite
prevalence
of
ER
.
Our
findings
therefore
warrant
further
study
of
PR
function
in
LAM
.