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Our Team
A New Clinically Based Staging System for Perihilar Cholangiocarcinoma.
[cholangiocarcinoma]
OBJECTIVES
:
Current
staging
systems
for
perihilar
cholangiocarcinoma
(
pCCA
)
are
inadequate
,
as
they
are
based
on
surgical
pathology
and
therefore
not
relevant
to
unresectable
patients
.
Clinical
trials
for
potential
targeted
therapies
for
pCCA
are
hampered
by
the
lack
of
an
accurate
,
nonoperative
staging
system
for
predicting
survival
.
We
aimed
at
developing
a
clinical
staging
system
for
pCCA
,
which
would
be
of
prognostic
relevance
for
all
pCCA
patients
and
help
stratify
patients
for
clinical
trials
.
METHODS
:
Clinical
information
at
the
time
of
pCCA
diagnosis
of
413
patients
seen
at
Mayo
Clinic
,
Rochester
,
MN
between
2002
and
2010
was
retrospectively
analyzed
.
A
survival
predictive
model
was
developed
using
Cox
proportional
hazards
analysis
.
The
performance
of
the
staging
system
was
compared
with
the
current
AJCC
/
UICC
(
the
American
Joint
Committee
on
Cancer
/
the
Union
for
International
Cancer
Control
)
7
th
tumor
-node-metastasis
(
TNM
)
staging
system
.
RESULTS
:
Eastern
Cooperative
Oncology
Group
(
ECOG
)
status
,
tumor
size
and
number
,
vascular
encasement
,
lymph
node
and
peritoneal
metastasis
and
CA
19
-
9
level
were
grouped
into
a
four
-tier
staging
system
.
The
median
survivals
of
stages
I
,
II
,
III
,
and
IV
patients
were
48
.
6
,
21
.
8
,
8
.
6
,
and
2
.
8
months
,
with
hazard
ratios
(
95
%
confidence
interval
)
of
1
.
0
(
reference
)
,
1
.
7
(
1
.
1
-
2
.
6
)
,
3
.
1
(
2
.
0
-
4
.
7
)
,
and
8
.
7
(
5
.
2
-
14
.
5
)
,
respectively
(
P
<
0
.
0001
)
.
This
staging
system
had
greater
concordance
statistics
(
standard
error
)
than
the
TNM
staging
system
(
0
.
725
(
0
.
018
)
vs
.
0
.
614
(
0
.
017
)
)
,
indicating
better
performance
in
predicting
survival
.
CONCLUSIONS
:
This
staging
system
,
based
on
nonoperative
information
at
the
time
of
pCCA
diagnosis
,
has
excellent
discriminatory
power
to
classify
patients
into
four
prognostic
stages
.
It
could
be
useful
to
clinicians
and
for
the
design
of
clinical
trials
.
Am
J
Gastroenterol
advance
online
publication
,
11
November
2014
;
doi
:
10
.
1038
/
ajg
.
2014
.
327
.
Diseases
Validation
Diseases presenting
"cancer"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
cadasil
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital adrenal hyperplasia
congenital diaphragmatic hernia
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
pleomorphic liposarcoma
primary effusion lymphoma
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
severe combined immunodeficiency
sneddon syndrome
systemic capillary leak syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated