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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
"tumor size"
symptom
adrenal incidentaloma
alpha-thalassemia
aniridia
carcinoma of the gallbladder
cholangiocarcinoma
dedifferentiated liposarcoma
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
liposarcoma
pleomorphic liposarcoma
von hippel-lindau disease
well-differentiated liposarcoma
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