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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 cells"
symptom
alpha-thalassemia
carcinoma of the gallbladder
cholangiocarcinoma
cushing syndrome
dedifferentiated liposarcoma
dentin dysplasia
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
junctional epidermolysis bullosa
kindler syndrome
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
severe combined immunodeficiency
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
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