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Surgical resection of sporadic and hereditary hemangioblastoma: Our 10-year experience and a literature review.
[von hippel-lindau disease]
Hemangioblastomas
(
HBLs
)
are
benign
neoplasms
that
contribute
to
1
-
2
.
5
%
of
intracranial
tumors
and
7
-
12
%
of
posterior
fossa
lesions
in
adult
patients
.
HBLs
either
evolve
hereditarily
in
association
with
von
Hippel-
Lindau
disease
(
vHL
)
or
,
more
prevalently
,
as
solitary
sporadic
tumors
.
Only
few
authors
have
reported
on
the
clinical
presentation
and
the
neurological
outcome
of
HBL
.
We
retrospectively
analyzed
the
clinical
,
radiological
,
surgical
,
and
histopathologic
records
of
24
consecutive
patients
(
11
men
,
13
women
;
mean
age
51
.
3
years
)
with
HBL
of
the
posterior
cranial
fossa
,
who
had
been
treated
at
our
center
between
2001
and
2012
.
We
reviewed
the
current
literature
,
and
discussed
our
findings
in
the
context
of
previous
publications
on
HBL
.
The
study
protocol
was
approved
by
the
local
ethics
committee
(
14
-
101
-
0070
)
.
Mean
time
to
diagnosis
was
14
weeks
.
The
extent
of
resection
(
EOR
)
was
total
in
20
and
near
total
in
4
patients
.
Four
patients
required
revision
within
24
h
because
of
relevant
postoperative
bleeding
.
One
patient
died
within
14
days
.
One
patient
required
permanent
shunting
.
At
discharge
,
75
%
of
patients
[
n
=
18
,
modified
Rankin
scale
(
mRS
)
0
-
1
]
showed
no
or
at
least
resolved
symptoms
.
Mean
follow-up
was
21
months
.
Two
recurrences
were
detected
during
follow-up
.
In
comparison
to
other
benign
entities
of
the
posterior
fossa
,
time
to
diagnosis
was
significantly
shorter
for
HBL
.
This
finding
indicates
the
rather
aggressive
biological
behavior
of
these
excessively
vascularized
tumors
.
In
our
series
,
however
,
the
rate
of
complete
resection
was
high
,
and
morbidity
and
mortality
rates
were
within
the
reported
range
.
Diseases
Validation
Diseases presenting
"excessively vascularized tumors"
symptom
von hippel-lindau disease
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