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Long-term Prognosis of Resected Pancreatic Neuroendocrine Tumors in von Hippel-Lindau Disease Is Favorable and Not Influenced by Small Tumors Left in Place.
[von hippel-lindau disease]
Management
of
pancreatic
neuroendocrine
tumors
(
PNETs
)
associated
with
von
Hippel-
Lindau
disease
(
VHL
)
is
challenging
because
of
the
malignant
potential
and
difficulty
in
predicting
prognosis
.
Compare
the
long
-term
outcome
of
resected
VHL
-PNET
and
sporadic
PNET
.
Data
of
all
patients
with
VHL
(
n
=
23
)
operated
on
for
nonmetastatic
PNET
were
reviewed
.
Patient
characteristics
and
recurrence-free
survival
rates
were
compared
with
those
in
patients
operated
on
for
sporadic
PNET
,
matched
for
tumor
size
,
stage
,
and
Ki-
67
index
.
Patients
in
both
groups
had
similar
demographic
characteristics
,
except
that
patients
with
VHL
were
younger
(
36
vs
56
years
,
P
<
0
.
0001
)
.
Median
tumor
size
was
30
mm
.
Median
Ki-
67
index
was
3
%
and
4
%
in
the
VHL
and
sporadic
groups
(
P
=
0
.
95
)
,
respectively
,
and
lymph
node
metastases
were
present
in
43
%
and
30
%
of
cases
,
respectively
(
P
=
0
.
45
)
.
Sixteen
(
70
%
)
patients
with
VHL
had
multiple
PNET
;
lesions
less
than
15
mm
were
left
in
place
in
11
patients
.
Median
postoperative
follow-up
was
107
months
(
interquartile
range
,
57
-
124
months
)
and
71
months
(
interquartile
range
,
58
-
131
months
)
in
the
VHL
and
control
groups
,
respectively
.
Median
recurrence-free
survival
could
not
have
been
estimated
in
the
VHL
group
due
to
the
low
number
of
events
(
hazard
ratio
,
5
.
6
;
95
%
confidence
interval
,
1
.
4
-
22
.
6
;
P
=
0
.
013
)
.
Five
patients
with
VHL
died
(
3
from
VHL
-related
tumors
including
1
from
PNET
)
,
whereas
only
one
control
patient
died
due
to
unrelated
causes
.
The
long
-term
outcome
of
resected
VHL
-PNET
is
better
than
that
of
sporadic
PNET
.
PNET
less
than
15
mm
left
in
place
did
not
progress
.
A
parenchyma-sparing
surgical
strategy
seems
appropriate
in
patients
with
VHL
-PNET
,
who
may
develop
more
life-threatening
tumors
of
other
organs
.
Diseases
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Diseases presenting
"except that patients with vhl were younger"
symptom
von hippel-lindau disease
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