Let's move on to our next case.
A 68-year-old woman with a history
of primary sclerosing cholangitis
presents to her primary care physician with 3
months of jaundice, weight loss and abdominal pain.
She has had a dull ache in her right
upper quadrant for the past few months.
Vitals are normal.
Physical exam reveals jaundice,
scleral icterus, diffuse muscle wasting
and tenderness to palpation
in the right upper quadrant.
Her lab studies are notable for AST of 99
(U/L), ALT 89 (U/L), total bilirubin 8.2 (mg/dL)
and alkaline phosphatase 89 (U/L).
A serum CA19-9 is elevated.
An abdominal ultrasound shows a
dilated intra and extrahepatic bile ducts,
hepatomegaly but no obvious hepatic masses.
What is the best next step
to confirm the diagnosis?
So let's point out some key things here.
She has jaundice, weight loss
and right upper quadrant pain
and know that she has a history of PSC.
Her physical exam and lab findings are consistent
with a cholestatic picture leading to jaundice.
And we're told that she has an elevated CA19-9 which
is a type of tumor marker, with dilated biliary ducts.
So, those may be clues to
help us figure out the diagnosis.
So now let's speak a bit
This is a cancer that is
specific to the bile duct.
It can originate anywhere along the biliary system
so it can be intrahepatic - within the liver,
or along the extrahepatic
ducts - outside of the liver.
It is the second most common
primary cancer of the liver after HCC,
and patients may come in
with right upper quadrant pain.
They may have constitutional symptoms
of generalized fatigue, and weight loss
and they may have jaundice.
The diagnosis is mostly by imaging
which can be done with MRCP or CT scan.
In addition, in unclear
cases, you might do an ERCP
and take brushings of the bile duct or
biopsy to help you confirm the diagnosis.
A serum CA 19-9 is a tumor
marker that you can check,
which may be elevated but know that this
cannot help you confirm or exclude the diagnosis.
Where it can be helpful, is in
tracking response to treatment
and whether or not patients have
experienced remission or relapse.
So there are several risk
factors for cholangiocarcinoma.
The first one, is a history of
primary sclerosing cholangitis.
Patients who have choledochal
cysts may also be at risk.
Rarely, liver fluke infections
can cause this cancer.
They may have prior
exposure to thorium dioxide
and stones within the liver, so hepatolithiasis
can also predispose patients to this condition.
So the treatment depends on
how extensive the disease is.
Usually, if the disease is limited to
the distal bile ducts outside of the liver,
you may do surgical resection.
For those lesions that are non-resectable, either
they involve structures around the biliary tree
and have invaded elsewhere,
you may do chemotherapy.
For those patients who then develop obstruction
from the cancer itself of the bile duct,
we may offer endoscopic stenting.
So here you can see first an example of
an MRCP showing a stricture in the bile duct,
And panel B, you now know see an ERCP with a scope
that has been advanced and this is prior to stenting.
And in panel C, you now see a stent being
placed and adequate flow through the biliary tree.
Lastly, the last treatment option we have is liver
transplantation when disease is non-resectable,
if it is less than 3 cm in size and
has not spread beyond the liver.
So, unfortunately, cholangiocarcinoma
is often a fatal diagnosis.
By the time most patient presents,
because they have such a vague symptoms,
they often have disease
that is no longer resectable.
So now, let's return to our case.
Our 68-year-old woman with a history of PSC,
is now coming in with jaundice and weight loss,
as well as right upper quadrant pain.
Her physical exam and labs are
consistent with a cholestatic picture
and her elevated CA19-9 is a hint that there
may be something going on related to malignancy.
So, that in addition to her
bile ducts on the ultrasound
should make you suspicious
and the best next step to confirm the
diagnosis is an ERCP for the biopsy to confirm.