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A rare case of peripheral nerve hemangioblastoma-case report and literature review.
[von hippel-lindau disease]
Hemangioblastoma
is
a
rare
benign
tumor
that
consists
of
neoplastic
vacuolated
stromal
cells
and
highly
developed
capillary
blood
vessels
.
These
tumors
are
almost
exclusively
found
in
the
central
nervous
system
,
but
in
extremely
rare
cases
,
it
can
arise
in
peripheral
nerves
.
In
the
majority
of
cases
,
it
occurs
sporadically
(
60
to
75
Â
%
)
,
but
in
about
25
Â
%
of
cases
,
it
is
associated
with
von
Hippel-
Lindau
disease
.
We
present
the
first
case
of
ulnar
nerve
hemangioblastoma
in
a
70
-
year
-old
male
.
The
patient
presented
with
a
slow
-growing
palpable
mass
on
the
front
side
of
the
right
upper
arm
.
Macroscopically
,
the
tumor
was
round
shaped
,
encapsulated
,
reddish-orange
in
color
,
very
well
vascularized
,
and
about
3
Â
cm
in
diameter
;
one
nerve
fascicle
was
entering
the
tumor
tissue
,
so
it
was
resected
with
the
tumor
.
The
rest
of
the
nerve
fascicles
were
intact
.
Postoperative
course
was
uneventful
.
Histopathological
analysis
with
immunohistochemical
analysis
confirmed
that
the
tumor
was
a
peripheral
nerve
hemangioblastoma
WHO
grade
I
.
Physical
treatment
was
conducted
,
and
there
was
no
motor
neurological
deficit
on
follow-up
after
3
Â
months
,
only
hypoesthesia
of
the
fourth
and
fifth
finger
.
These
lesions
are
so
rarely
found
arising
from
peripheral
nerves
that
only
four
published
cases
exist
in
literature
today
.
There
is
very
little
data
about
these
tumors
in
world
literature
,
so
we
consider
our
paper
a
valuable
contribution
to
global
knowledge
on
these
tumors
.
Diseases
Validation
Diseases presenting
"hemangioblastoma"
symptom
liposarcoma
von hippel-lindau disease
This symptom has already been validated