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PAX8 expression in sporadic hemangioblastoma of the kidney supports a primary renal cell lineage: implications for differential diagnosis.
[von hippel-lindau disease]
Hemangioblastoma
is
a
benign
,
morphologically
distinctive
neoplasm
of
disputed
histogenesis
that
typically
occurs
in
the
central
nervous
system
either
in
the
setting
of
von
Hippel-
Lindau
disease
or
more
often
sporadically
.
Extraneural
hemangioblastoma
is
exceptional
and
raises
a
challenging
differential
diagnosis
.
Herein
,
we
report
a
primary
renal
hemangioblastoma
occurring
in
51
-
year
-old
woman
without
stigmata
of
von
Hippel-
Lindau
disease
.
Histologically
,
the
tumor
was
composed
of
sheets
of
polygonal
epithelioid
stromal
cells
with
ample
pale
or
eosinophilic
,
vacuolated
cytoplasm
in
an
arborizing
capillary
network
.
Tumor
cells
showed
variable
nuclear
pleomorphism
,
intranuclear
cytoplasmic
invaginations
,
scattered
hyaline
globules
,
and
psammoma-like
calcifications
.
Some
areas
showed
branching
hemangiopericytoma-like
vessels
with
tumor
cells
radiating
from
the
wall
,
while
other
areas
were
edematous
and
hyalinized
with
sparse
stromal
cells
and
abundant
reticular
vessels
.
Immunohistochemically
,
the
tumor
cells
reacted
strongly
and
diffusely
with
antibodies
to
PAX
8
,
CD
10
,
α-inhibin
,
S
100
protein
,
neuron-
specific
enolase
,
and
vimentin
,
and
they
showed
focal
positivity
with
antibodies
to
epithelial
membrane
antigen
and
AE
1
/
AE
3
.
Tumor
cells
were
negative
for
CK
7
,
CK
8
/
18
,
RCC
antigen
,
synaptophysin
,
chromogranin
,
c-kit
,
D
2
-
40
,
HMB
45
,
melan-
A
,
cathepsin
K
,
SMA
,
desmin
,
CD
31
,
CD
34
,
and
estrogen
and
progesterone
receptors
.
Positive
immunoreactivity
for
PAX
8
is
unexpected
and
contrasts
to
central
nervous
system
(
CNS
)
hemangioblastomas
,
which
are
essentially
always
negative
for
PAX
8
.
This
novel
finding
adds
support
to
the
hypothesis
that
the
immunoprofile
of
extraneural
hemangioblastoma
varies
with
site
of
origin
,
perhaps
as
a
result
of
tumor
cell
lineage
and
retention
of
organ-
specific
markers
or
acquisition
of
site-
specific
antigens
due
to
local
factors
.
Diseases
Validation
Diseases presenting
"specific markers"
symptom
cutaneous mastocytosis
monosomy 21
von hippel-lindau disease
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