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Renal tumor size is an independent prognostic factor for overall survival in von Hippel-Lindau disease.
[von hippel-lindau disease]
To
evaluate
the
effect
of
renal
cell
carcinoma
(
RCC
)
on
survival
in
von
Hippel-
Lindau
(
VHL
)
disease
and
to
assess
the
relationship
between
tumor
size
and
survival
.
In
this
retrospective
cohort
study
,
the
medical
records
of
72
patients
who
presented
with
VHL
disease
between
1994
and
2012
were
reviewed
.
Clinical
VHL
-related
characteristics
were
analyzed
,
and
the
prognostic
value
of
renal
tumor
size
for
overall
survival
was
assessed
by
using
Cox
regression
models
.
Of
the
72
VHL
patients
,
42
(
58
.
3
%
)
and
30
(
41
.
7
%
)
were
male
and
female
,
respectively
.
The
mean
age
was
37
.
9
years
,
and
the
median
follow-up
period
was
61
.
5
months
.
In
terms
of
VHL
-related
manifestations
,
40
(
55
.
6
%
)
had
RCC
,
46
(
63
.
8
%
)
had
hemangioblastoma
in
the
cerebellum
,
10
(
13
.
9
%
)
had
hemangioblastoma
in
the
spinal
cord
,
34
(
47
.
2
%
)
had
a
pancreatic
mass
,
18
(
25
.
0
%
)
had
pheochromocytoma
,
and
14
(
19
.
4
%
)
had
retinal
capillary
hemangioma
.
RCC
was
a
major
cause
of
mortality
:
Of
the
11
patients
who
died
,
nine
(
12
.
5
%
)
died
due
to
RCC
progression
.
The
5
-
year
overall
survival
rate
was
85
.
6
%
for
all
patients
,
96
.
9
%
for
patients
without
RCC
,
83
.
6
%
for
patients
with
RCC
<
3
cm
,
and
75
.
8
%
for
patients
with
RCC
≥
3
cm
.
Multivariable
analysis
showed
that
RCC
≥
3
cm
was
an
independent
predictor
of
overall
survival
(
HR
9
.
87
,
95
%
CI
1
.
17
-
83
.
00
,
p
=
0
.
035
)
along
with
age
(
HR
1
.
05
,
95
%
CI
1
.
01
-
1
.
10
,
p
=
0
.
027
)
.
Renal
tumor
size
was
an
independent
prognostic
factor
for
overall
survival
in
VHL
disease
.
This
observation
will
be
helpful
for
planning
RCC
treatment
in
VHL
disease
.
Diseases
Validation
Diseases presenting
"independent prognostic factor"
symptom
carcinoma of the gallbladder
cholangiocarcinoma
cutaneous mastocytosis
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
severe combined immunodeficiency
von hippel-lindau disease
waldenström macroglobulinemia
wiskott-aldrich syndrome
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