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The significance of genetics for cholangiocarcinoma development.
[cholangiocarcinoma]
Cholangiocarcinoma
(
CCA
)
is
a
rare
malignancy
of
the
liver
,
arising
from
bile
ducts
.
The
incidence
is
increasing
worldwide
,
but
the
prognosis
has
remained
dismal
and
virtually
unchanged
in
the
past
30
years
.
Although
several
risk
factors
have
been
associated
with
the
development
of
this
cancer
,
none
of
them
are
normally
identified
in
most
patients
.
Diagnosis
in
advanced
stages
of
the
disease
and
limited
therapeutic
options
contribute
to
poor
survival
rates
.
The
recent
analysis
of
genetic
and
epigenetic
alterations
occurring
in
CCA
has
shed
new
light
in
the
understanding
of
the
molecular
mechanisms
leading
to
the
malignant
transformation
of
biliary
cells
.
Further
studies
in
this
direction
may
foster
new
diagnostic
,
prognostic
and
therapeutic
approaches
.
This
review
provides
a
global
overview
of
recent
advances
in
CCA
and
describes
the
most
important
genetic
mutations
and
epigenetic
alterations
so
far
reported
in
CCA
.
