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Lymphatic endothelial differentiation in pulmonary lymphangioleiomyomatosis cells.
[lymphangioleiomyomatosis]
Pulmonary
lymphangioleiomyomatosis
(
LAM
)
is
a
rare
,
low
-grade
neoplasm
affecting
almost
exclusively
women
of
childbearing
age
.
LAM
belongs
to
the
family
of
perivascular
epithelioid
cell
tumors
,
characterized
by
spindle
and
epithelioid
cells
with
smooth
muscle
and
melanocytic
differentiation
.
LAM
cells
infiltrate
the
lungs
,
producing
multiple
,
bilateral
lesions
rich
in
lymphatic
channels
and
forming
cysts
,
leading
to
respiratory
insufficiency
.
Here
we
used
antibodies
against
four
lymphatic
endothelial
markers-
podoplanin
(
detected
by
D
2
-
40
)
,
prospero
homeobox
1
(
PROX
1
)
,
vascular
endothelial
growth
factor
receptor
3
(
VEGFR-
3
)
,
and
lymphatic
vessel
endothelial
hyaluronan
receptor
1
(
LYVE
1
)
-
to
determine
whether
LAM
cells
show
lymphatic
differentiation
.
Twelve
of
12
diagnostic
biopsy
specimens
(
early
-
stage
LAM
)
and
19
of
19
explants
(
late
-
stage
LAM
)
showed
immunopositivity
for
D
2
-
40
in
most
neoplastic
cells
.
PROX
1
,
VEGFR-
3
,
and
LYVE
1
immunoreactivity
varied
from
scarce
in
the
early
stage
to
abundant
in
the
late
stage
.
Lymphatic
endothelial
,
smooth
muscle
,
and
melanocytic
markers
were
partially
co
-
localized
.
These
findings
indicate
that
lymphatic
endothelial
differentiation
is
a
feature
of
LAM
and
provide
evidence
of
a
previously
unidentified
third
lineage
of
differentiation
in
this
neoplasm
.
This
study
has
implications
for
the
histological
diagnosis
of
LAM
,
the
origin
of
the
neoplastic
cells
,
and
potential
future
treatment
with
drugs
targeting
lymphangiogenesis
.
Diseases
Validation
Diseases presenting
"growth factor receptor"
symptom
achondroplasia
aromatase deficiency
cholangiocarcinoma
dedifferentiated liposarcoma
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
kallmann syndrome
lymphangioleiomyomatosis
oral submucous fibrosis
proteus syndrome
severe combined immunodeficiency
wiskott-aldrich syndrome
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