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The risk of carcinogenesis in congenital choledochal cyst patients: an analysis of 214 cases.
[cholangiocarcinoma]
Background
.
Choledochal
cysts
are
most
common
in
Asian
populations
.
In
addition
,
the
incidence
of
biliary
tract
malignancies
from
choledochal
cysts
is
increasing
,
but
the
risk
of
carcinogenesis
is
still
unclear
.
Material
and
methods
.
Clinical
data
from
214
congenital
choledochal
cyst
cases
from
1968
to
2013
were
retrospectively
analyzed
.
Results
.
Todani
type
I
was
more
common
(
139
,
65
.
0
%
)
than
type
IVa
(
53
,
24
.
8
%
)
or
type
V
(
17
,
7
.
9
%
)
in
these
choledochal
cyst
patients
.
Biliary
tract
malignant
tumors
occurred
in
the
gallbladder
(
2
,
13
.
3
%
)
,
common
bile
duct
(
10
,
66
.
7
%
)
,
and
intrahepatic
bile
duct
(
3
,
20
%
)
in
15
patients
(
7
.
0
%
)
,
including
one
patient
in
whom
malignant
transformation
occurred
in
the
intrahepatic
bile
duct
in
a
type
IVa
patient
15
years
after
extrahepatic
cyst
resection
.
An
age
at
symptom
onset
≥
60
years
was
a
risk
factor
(
p
<
0
.
001
)
,
while
an
initial
complete
surgery
was
a
protective
factor
for
carcinogenesis
(
p
=
0
.
001
)
.
Conclusions
.
Choledochal
cysts
should
be
removed
once
diagnosed
because
of
an
increased
risk
of
malignant
transformation
with
increasing
age
.
Complete
cyst
removal
is
necessary
for
the
first
surgical
treatment
.
Additional
hepatectomy
should
be
considered
for
type
IVa
choledochal
cysts
because
cholangiocarcinoma
can
arise
from
the
intrahepatic
bile
duct
years
after
the
extrahepatic
cyst
excision
.
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
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