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Spectrum of magnetic resonance imaging findings in pancreatic and other abdominal manifestations of Von Hippel-Lindau disease in a series of 23 patients: a pictorial review.
[von hippel-lindau disease]
Von
Hippel
Lindau
disease
is
a
rare
autosomal
dominantly
inherited
multisystem
disorder
characterized
by
development
of
benign
and
malignant
tumors
.
The
abdominal
manifestation
of
the
syndrome
are
protean
.
Magnetic
resonance
plays
an
important
role
in
identification
of
abdominal
abnormalities
and
follow-up
of
lesions
.
To
describe
magnetic
resonance
imaging
findings
and
patterns
of
pancreatic
and
other
principal
abdominal
manifestations
in
a
series
of
von
Hippel-
Lindau
(
VHL
)
disease
patients
and
to
review
literature
.
We
retrospectively
reviewed
abdominal
magnetic
resonance
studies
performed
in
23
patients
(
10
males
,
13
females
)
diagnosed
of
VHL
.
In
all
examined
patients
abdominal
involvement
was
present
.
The
pancreatic
imaging
findings
detected
were
:
unilocular
cystic
lesions
(
6
/
23
:
26
.
1
%
)
;
serous
cystadenomas
(
11
/
23
:
47
.
8
%
)
,
including
diffuse
lesions
(
8
/
23
:
34
.
8
%
)
;
solid
neuroendocrine
tumors
(
8
/
23
:
34
.
8
%
)
;
cystic
neuroendocrine
tumors
(
1
/
23
:
4
.
3
%
)
.
The
renal
findings
detected
were
:
simple
renal
cysts
(
18
/
23
:
78
.
3
%
)
;
complex
renal
cysts
(
13
/
23
:
56
.
5
%
)
,
including
benign
lesions
(
10
/
23
:
43
.
5
%
)
and
malignant
lesions
(
3
/
23
:
13
.
0
%
)
;
renal
carcinomas
(
11
/
23
:
47
.
8
%
)
and
5
of
these
(
45
.
5
%
)
were
multiple
and
bilateral
.
Five
patients
(
21
.
7
%
)
presented
pheochromocytoma
(
4
of
these
were
bilateral
;
80
.
0
%
)
and
1
patient
(
4
.
3
%
)
presented
cystadenoma
of
the
epididymis
.
In
VHL
disease
patients
,
magnetic
resonance
imaging
plays
an
essential
role
in
the
identification
of
pancreatic
and
other
abdominal
lesions
,
in
their
follow-up
,
in
the
screening
of
asymptomatic
gene
carriers
,
and
in
their
long
-term
surveillance
.