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Incidental perivascular epithelioid cell tumor in an inguinal hernia sac.
[lymphangioleiomyomatosis]
Perivascular
epithelioid
tumors
(
PEComa
)
are
uncommon
mesenchymal
neoplasms
demonstrating
positivity
for
muscular
and
melanocytic
immuno-markers
.
Included
in
this
category
are
angiomyolipoma
,
lymphangioleiomyomatosis
,
and
clear
cell
sugar
tumors
.
Lesions
which
do
not
fit
into
these
categories
are
classified
as
"
not
otherwise
specified
"
.
We
present
a
case
of
an
incidentally
discovered
PEComa
within
inguinal
hernia
sac
contents
in
a
70
-
year
-old
woman
.
It
consisted
of
spindled
and
epithelioid
cells
with
bland
oval
nuclei
,
small
nucleoli
and
clear
to
light
eosinophilic
cytoplasm
.
There
was
no
atypia
or
mitoses
.
The
lesion
was
strongly
positive
for
HMB
45
and
smooth
muscle
actin
.
Pelvic
soft
tissue
and
peritoneal
PEComas
are
rarely
reported
in
literature
and
very
little
is
known
about
their
prognosis
.
We
discuss
the
immunohistochemistry
,
differential
diagnosis
,
and
pathogenesis
of
PEComas
.
Diseases
Validation
Diseases presenting
"peritoneal pecomas are rarely reported in literature and very little is known about their prognosis"
symptom
lymphangioleiomyomatosis
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