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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
Validation
Diseases presenting
"long-term outcome"
symptom
aniridia
cholangiocarcinoma
classical phenylketonuria
cystinuria
erythropoietic protoporphyria
esophageal squamous cell carcinoma
fabry disease
hirschsprung disease
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
kabuki syndrome
krabbe disease
oligodontia
papillon-lefèvre syndrome
primary hyperoxaluria type 1
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
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