Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
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
.
Diseases
Validation
Diseases presenting
"malignant tumors"
symptom
adrenal incidentaloma
cholangiocarcinoma
coats disease
cowden syndrome
cushing syndrome
dedifferentiated liposarcoma
dentinogenesis imperfecta
esophageal carcinoma
focal myositis
junctional epidermolysis bullosa
liposarcoma
pleomorphic liposarcoma
von hippel-lindau disease
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom