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A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina.
[lymphangioleiomyomatosis]
Perivascular
epithelioid
cell
tumors
(
PEComas
)
are
a
family
of
rare
mesenchymal
neoplasms
,
including
angiomyolipoma
,
clear-cell
"
sugar
"
tumor
of
the
lung
and
extrapulmonary
sites
,
lymphangioleiomyomatosis
,
clear-cell
myomelanocytic
tumor
of
the
falciform
ligament
/
ligamentum
teres
,
and
clear-cell
tumors
at
various
other
anatomic
sites
.
These
tumors
are
characterized
by
a
proliferation
of
epithelioid
cells
with
clear
to
eosinophilic
cytoplasm
,
perivascular
distribution
,
and
coexpression
of
myogenic
and
melanocytic
markers
.
PEComas
show
a
female
predominance
,
occur
with
some
frequency
in
the
gynecological
tract
,
and
have
an
unpredictable
biological
behavior
.
We
report
a
case
of
a
huge
malignant
PEComa
arising
from
the
uterine
cervix
and
vagina
.
To
the
best
of
our
knowledge
,
only
6
cases
of
PEComa
in
the
cervix
and
2
cases
in
the
vagina
have
been
reported
in
the
literature
.
Diseases
Validation
Diseases presenting
"extrapulmonary sites"
symptom
lymphangioleiomyomatosis
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