Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature.
[lymphangioleiomyomatosis]
The
perivascular
epithelioid
cell
tumor
(
PEComa
)
family
of
tumors
includes
angiomyolipoma
,
lymphangioleiomyomatosis
,
clear
cell
sugar
tumor
of
the
lung
,
clear
cell
myomelanocytic
tumor
of
the
falciform
ligament
/
ligamentum
teres
,
and
rare
clear
cell
tumors
of
other
anatomical
sites
(
PEComas-NOS
)
.
Among
the
PEComas-NOS
,
pigmented
variants
are
extremely
rare
.
The
case
concerns
a
50
-
year
-old
female
who
presented
with
pain
in
right
hypochondrium
.
The
resected
specimen
included
a
24
×
18
×
9
cm
mass
.
The
tumor
was
histologically
characterized
by
both
spindle
and
epithelioid
cells
with
round
to
oval
nuclei
and
clear
to
eosinophilic
cytoplasm
containing
abundant
melanin
pigment
.
The
stroma
demonstrated
intervening
,
thin
,
fibrovascular
septa
.
Immunohistochemically
,
the
tumor
cells
were
strongly
positive
for
HMB-
45
,
weak
positive
for
smooth
muscle
actin
(
SMA
)
,
and
negative
for
Hep
Par
1
,
Glypican
3
,
MUM-
1
,
and
S-
100
protein
.
The
patient
had
no
evidence
of
disease
24
months
after
surgery
.
Pathologists
and
clinicians
should
know
about
the
existence
of
pigmented
perivascular
epithelioid
cell
tumor
of
the
liver
.
Diseases
Validation
Diseases presenting
"tumor of the lung"
symptom
lymphangioleiomyomatosis
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom