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Outcomes in biliary malignancy.
[cholangiocarcinoma]
The
biliary
malignancies
that
are
reviewed
here
are
gallbladder
cancer
(
GBC
)
,
intrahepatic
cholangiocarcinoma
(
IHC
)
,
and
perihilar
cholangiocarcinoma
(
PHC
)
.
The
focus
is
on
outcomes
after
potentially
curative
resection
of
biliary
malignancies
.
Key
outcomes
are
postoperative
mortality
,
median
and
5
-
year
overall
survival
(
OS
)
,
recurrence-free
survival
,
and
recurrence
patterns
.
Poor
prognostic
factors
for
recurrence
and
survival
as
well
as
prognostic
models
are
also
discussed
.
The
incidence
of
biliary
malignancies
in
the
United
States
is
about
5
in
100
,
000
.
Postoperative
mortality
for
resection
of
GBC
and
IHC
is
similar
to
that
of
liver
resections
for
other
indications
.
However
,
90
day
postoperative
mortality
after
liver
resection
for
PHC
is
about
10
%
.
For
GBC
,
median
OS
depends
strongly
on
the
T
-
stage
and
ranges
from
8
months
(
pT
3
)
to
79
months
(
pT
1
b
)
.
Median
OS
after
resection
for
IHC
is
about
30
months
,
and
for
PHC
about
38
months
.
The
majority
of
patients
with
biliary
malignancies
develop
a
recurrence
after
resection
.
Patients
with
GBC
recur
early
with
a
median
time
to
recurrence
of
12
months
,
versus
about
20
months
for
IHC
and
PHC
.
In
patients
with
resected
IHC
or
PHC
locoregional
recurrence
was
the
only
site
of
recurrence
in
about
60
%
of
patients
,
versus
15
%
in
patients
with
GBC
.
Poor
prognostic
factors
after
resection
of
all
biliary
malignancies
include
the
presence
of
lymph
node
metastasis
,
a
positive
surgical
resection
margin
,
and
moderate
or
poor
tumor
differentiation
.
Several
prognostic
nomograms
have
been
developed
to
predict
long
-term
outcomes
of
biliary
cancer
resection
.
Diseases
Validation
Diseases presenting
"long-term outcomes"
symptom
acute rheumatic fever
alpha-thalassemia
aromatase deficiency
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cushing syndrome
dystrophic epidermolysis bullosa
esophageal squamous cell carcinoma
hirschsprung disease
homocystinuria without methylmalonic aciduria
kabuki syndrome
lamellar ichthyosis
omenn syndrome
phenylketonuria
proteus syndrome
trochlear dysplasia
von hippel-lindau disease
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