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Histomorphological spectrum and immunohistochemical characterization of hemangioblastomas: An entity of unclear histogenesis.
[von hippel-lindau disease]
Hemangioblastomas
(
HBs
)
are
rare
WHO
grade
I
neoplasms
of
uncertain
histogenesis
.
Most
are
sporadic
and
association
with
von
Hippel-
Lindau
disease
(
VHL
)
is
uncommon
.
Histomorphological
and
immunohistochemical
evaluation
of
24
cases
of
HBs
was
done
.
Age
range
was
15
-
68
yrs
(
median
:
30
yrs
)
with
male
:
Female
of
1
.
2
:
1
(
M-
13
;
F-
11
)
.
Cerebellum
was
commonest
location
(
n
=
20
)
,
one
each
was
seen
in
brain
stem
,
cervical
spinal
cord
,
fourth
ventricle
and
frontal
lobe
,
respectively
.
VHL
association
was
noted
in
5
cases
.
Four
cases
were
recurrent
in
nature
of
which
3
were
in
association
with
vHL
.
Histologically
,
reticular
variant
was
the
predominant
subtype
(
n
=
15
)
,
5
were
of
cellular
variant
and
4
were
mixed
.
Nuclear
pleomorphism
,
nuclear
cytoplasmic
inclusions
,
cytoplasmic
vacuolation
were
noted
in
the
stromal
cells
in
varying
proportions
.
Immunohistochemical
evaluation
was
successful
in
only
11
cases
and
of
which
8
showed
stromal
cell
positivity
for
alpha-inhibin
.
CD
5
6
(
NCAM
)
,
Nestin
and
synaptophysin
positivity
was
seen
in
6
,
7
and
4
cases
,
respectively
.
Nestin
positivity
was
noted
in
stromal
cells
only
and
no
reactivity
with
the
endothelial
cells
seen
.
S-
100
protein
and
NSE
positivity
was
seen
in
8
and
10
cases
,
respectively
.
Glial
fibrillary
acidic
protein
(
GFAP
)
showed
two
distinct
patterns
of
immunoreactivity
-
scattered
stromal
cell
positivity
(
n
:
5
)
and
pattern
of
reactive
astrogliosis
positivity
(
n
:
10
)
.
CD
44
positivity
was
noted
in
5
cases
.
VEGF
and
EGFR
positivity
was
seen
in
5
cases
each
.
None
of
the
cases
showed
positivity
for
epithelial
membrane
antigen
and
no
stromal
cells
in
any
of
the
cases
showed
positivity
for
CD
34
and
CD
31
.
HBs
can
occur
in
throughout
the
neuroaxis
.
Cerebellum
is
the
commonest
site
of
occurrence
for
HBs
and
uncommonly
can
occur
in
the
supratentorial
compartment
and
spinal
cord
.
Its
association
with
vHL
is
uncommon
and
no
histological
or
immunohistochemical
correlation
was
identified
with
the
same
.
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