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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 transformation"
symptom
achondroplasia
adrenal incidentaloma
child syndrome
cholangiocarcinoma
cutaneous mastocytosis
epidermolysis bullosa simplex
esophageal adenocarcinoma
liposarcoma
oral submucous fibrosis
severe combined immunodeficiency
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