Cholangiocarcinoma diagnosis and management the lect.ppt

Surgical Management of
Surgical Management of
Cholangiocarcinoma
Cholangiocarcinoma
HAMED RASHAD
HAMED RASHAD
Professor of surgery Banha faculty of medicine - Egypt
Professor of surgery Banha faculty of medicine - Egypt

 Drawings illustrate the Bismuth-Corlette classification of
Drawings illustrate the Bismuth-Corlette classification of
perihilar cholangiocarcinomas. Type I involves the
perihilar cholangiocarcinomas. Type I involves the
common hepatic duct (CHD); type II, the CHD and the
common hepatic duct (CHD); type II, the CHD and the
junction of the RHD and LHD; type IIIA, the CHD, biliary
junction of the RHD and LHD; type IIIA, the CHD, biliary
junction, and RHD; type IIIB, the CHD, biliary junction,
junction, and RHD; type IIIB, the CHD, biliary junction,
and LHD; and type IV, the CHD and the biliary junction,
and LHD; and type IV, the CHD and the biliary junction,
with extension to both the RHD and LHD or a multifocal
with extension to both the RHD and LHD or a multifocal
bile duct tumor.
bile duct tumor.

Bismuth-Corlette classification
Bismuth-Corlette classification
scheme for cholangiocarcinomas. MR
scheme for cholangiocarcinomas. MR
cholangiograms were obtained in five
different patients. Drawings of the lesions
different patients. Drawings of the lesions
are shown in the corresponding insets
are shown in the corresponding insets
Type I involves the CHD with abrupt
Type I involves the CHD with abrupt
cutoff of the RHD and LHD at the
cutoff of the RHD and LHD at the
confluence (arrow in
confluence (arrow in a
a); type II, the CHD
); type II, the CHD
(thick arrow in
(thick arrow in b
b) and the junction of the
) and the junction of the
RHD and LHD (thin arrow in
RHD and LHD (thin arrow in b
b); type
); type
IIIA, the CHD (arrow in
IIIA, the CHD (arrow in c
c), biliary
), biliary
junction, and RHD (arrowheads in
junction, and RHD (arrowheads in c
c);
);
type IIIB, the CHD (arrow in
type IIIB, the CHD (arrow in d
d), biliary
), biliary
junction, and LHD (arrowhead in
junction, and LHD (arrowhead in d
d); and
); and
type IV, the CHD (arrow in
type IV, the CHD (arrow in e
e) and the
) and the
biliary junction, with extension to both
the RHD and LHD (arrowheads in
the RHD and LHD (arrowheads in e
e) or a
) or a
multifocal bile duct tumor.
multifocal bile duct tumor.

MRI of a Mass-forming
MRI of a Mass-forming
cholangiocarcinoma
cholangiocarcinoma
(a)
(a) Out-of-phase gradient-echo
Out-of-phase gradient-echo
T1-weighted MR image shows a
T1-weighted MR image shows a
hypointense lobulated mass in the
hypointense lobulated mass in the
right hepatic lobe (arrows).
right hepatic lobe (arrows). (b)
(b) On
On
a fat-saturated T2-weighted MR
a fat-saturated T2-weighted MR
image, the mass appears
image, the mass appears
hyperintense (arrows).
hyperintense (arrows). (c)
(c) Early-
Early-
phase contrast-enhanced T1-
weighted MR image shows
irregular peripheral enhancement
irregular peripheral enhancement
of the mass (arrows).
of the mass (arrows). (d)
(d) Delayed
Delayed
progressive heterogeneous
progressive heterogeneous
enhancement of the lesion (
enhancement of the lesion (*
*).
).

Mass-forming
Mass-forming
cholangiocarcinoma arising
cholangiocarcinoma arising
in a cirrhotic liver.
in a cirrhotic liver. (a)
(a) MR
MR
image shows a slightly
image shows a slightly
hyperintense mass with
hyperintense mass with
capsular retraction (arrow)
capsular retraction (arrow)
in segment V of the liver.
in segment V of the liver.
(b)
(b) Contrast-enhanced MR
Contrast-enhanced MR
image shows strong
image shows strong
enhancement of the tumor
enhancement of the tumor
(arrow).
(arrow). (c)
(c)
Photomicrograph shows a
Photomicrograph shows a
fibrotic pseudocapsule
fibrotic pseudocapsule
(arrowheads) surrounding
(arrowheads) surrounding
the tumor (
the tumor (*
*).
).

Typical features of mass-forming cholangiocarcinoma
Typical features of mass-forming cholangiocarcinoma
at CT,
at CT, (a)
(a) Arterial phase CT scan shows a tumor with
Arterial phase CT scan shows a tumor with
ragged rim enhancement at the periphery (arrow).
ragged rim enhancement at the periphery (arrow). (b)
(b)
Axial portal venous phase CT scan shows gradual
Axial portal venous phase CT scan shows gradual
centripetal enhancement of the tumor with capsular
centripetal enhancement of the tumor with capsular
retraction (black arrow). A satellite nodule is also seen
retraction (black arrow). A satellite nodule is also seen
(white arrow).
(white arrow). (c)
(c) Three-minute delayed phase CT scan
Three-minute delayed phase CT scan
shows gradual centripetal enhancement with tumor
shows gradual centripetal enhancement with tumor
encasement of the posterior branch of the right portal
encasement of the posterior branch of the right portal
vein (arrowhead). Encasement of a portal or hepatic
vein (arrowhead). Encasement of a portal or hepatic
vein without formation of a grossly visible tumor
vein without formation of a grossly visible tumor
thrombus is one of the distinguishing features of
thrombus is one of the distinguishing features of
cholangiocarcinoma as opposed to HCC.
cholangiocarcinoma as opposed to HCC. (d)
(d)
Photograph of the gross specimen shows a
Photograph of the gross specimen shows a
homogeneous sclerotic mass with an irregular
homogeneous sclerotic mass with an irregular
infiltrative margin and a central area of whitish scarred
infiltrative margin and a central area of whitish scarred
tissue (
tissue (*
*), findings that correlate well with microscopic
), findings that correlate well with microscopic
findings, namely, tumor cells that are more prominent
findings, namely, tumor cells that are more prominent
at the periphery of the mass, with fibrotic stroma being
at the periphery of the mass, with fibrotic stroma being
more prominent in the center of the tumor.
more prominent in the center of the tumor. (e)
(e)
Photomicrograph ( of the periphery of the tumor
Photomicrograph ( of the periphery of the tumor
shows the indistinct tumor margin, in which tumor
shows the indistinct tumor margin, in which tumor
cells (
cells (*
*) are intermingled with normal hepatocytes in
) are intermingled with normal hepatocytes in
the adjacent liver (arrows).
the adjacent liver (arrows). (f)
(f) Photomicrograph of the
Photomicrograph of the
inner portion of the tumor shows a larger amount of
inner portion of the tumor shows a larger amount of
fibrous tissue with scattered tumor cells (arrows).
fibrous tissue with scattered tumor cells (arrows).

Cholangiocarcinoma diagnosis and management the lect.ppt

Metastatic inoperable cholangiocarcinoma in a 66-year-old woman who
presented with abdominal pain and jaundice. (a) FDG PET scan shows
increased uptake in a mass-forming cholangiocarcinoma in
the left hepatic lobe (arrow) and in liver metastases (arrowheads), findings
that prohibited surgery. (b) Followup FDG PET scan obtained after 2 months
of gemcitabine-avastin therapy shows significantly reduced uptake
in all the lesions, findings that signify a good response.

Intraductal cholangiocarcinoma in a 56-year-old woman with PSC.
Intraductal cholangiocarcinoma in a 56-year-old woman with PSC. (a)
(a) Coronal
Coronal
FDG PET scan shows increased uptake in an extrahepatic malignant biliary
FDG PET scan shows increased uptake in an extrahepatic malignant biliary
stricture (cholangiocarcinoma) (thin arrow). However, increased uptake is also
stricture (cholangiocarcinoma) (thin arrow). However, increased uptake is also
seen in intrahepatic benign strictures (thick arrow).
seen in intrahepatic benign strictures (thick arrow). (b)
(b) Corresponding ERC
Corresponding ERC
image shows a long extrahepatic stricture (thin arrow), which was confirmed to
image shows a long extrahepatic stricture (thin arrow), which was confirmed to
be malignant at biopsy, and a beaded appearance of the right intrahepatic duct
be malignant at biopsy, and a beaded appearance of the right intrahepatic duct
due to multiple benign strictures (thick
due to multiple benign strictures (thick arrow).
arrow).

Mass-forming cholangiocarcinoma in segment IV of the liver in a 73-year-old woman.
Mass-forming cholangiocarcinoma in segment IV of the liver in a 73-year-old woman.
On coronal subtracted images from dynamic contrast-enhanced 3D fat-saturated
On coronal subtracted images from dynamic contrast-enhanced 3D fat-saturated
gradient-echo MR imaging data obtained before
gradient-echo MR imaging data obtained before (a)
(a) and 6 weeks after
and 6 weeks after (b)
(b) the initiation
the initiation
of antiangiogenic drug treatment, the cholangiocarcinoma demonstrates peripheral
of antiangiogenic drug treatment, the cholangiocarcinoma demonstrates peripheral
heterogeneous enhancement. The tumor has been outlined to indicate a region of
heterogeneous enhancement. The tumor has been outlined to indicate a region of
interest for the calculation of vascularization parameters. The decrease in tumor
interest for the calculation of vascularization parameters. The decrease in tumor
vascularization after antiangiogenic drug administration, seen in
vascularization after antiangiogenic drug administration, seen in b
b, was confirmed with
, was confirmed with
transfer constant and extracellular volume fraction calculations.
transfer constant and extracellular volume fraction calculations.

Mass-forming cholangiocarcinoma in a 41-year-old man with jaundice and altered liver
Mass-forming cholangiocarcinoma in a 41-year-old man with jaundice and altered liver
function.
function.(a)
(a) Fat-saturated T2-weighted MR image shows a hyperintense mass in the left
Fat-saturated T2-weighted MR image shows a hyperintense mass in the left
hepatic lobe and extending to the porta hepatis (arrow), with dilatation of the biliary
hepatic lobe and extending to the porta hepatis (arrow), with dilatation of the biliary
radicles in both lobes (arrowheads).
radicles in both lobes (arrowheads). (b)
(b) Out-of-phase gradientecho T1-weighted MR
Out-of-phase gradientecho T1-weighted MR
image shows the mass encasing the left portal vein (arrow) and extending to the porta
image shows the mass encasing the left portal vein (arrow) and extending to the porta
hepatis.
hepatis. (c, d)
(c, d) Corresponding contrast-enhanced MR angiograms show encasement of the
Corresponding contrast-enhanced MR angiograms show encasement of the
left hepatic artery (arrow in
left hepatic artery (arrow in c
c)and invasion and nonvisualization of the left portal vein
)and invasion and nonvisualization of the left portal vein
(arrow in
(arrow in d
d), which prohibited surgery.
), which prohibited surgery.

CT appearances of cholangiocarcinoma. (a)
Coronal reformatted CT image obtained in a
Coronal reformatted CT image obtained in a
shows an infiltrating intraductal extrahepatic
shows an infiltrating intraductal extrahepatic
cholangiocarcinoma causing irregular thickening
cholangiocarcinoma causing irregular thickening
and enhancement (white arrows) of the CBD with
and enhancement (white arrows) of the CBD with
upstream dilatation of intrahepatic biliary radicles
upstream dilatation of intrahepatic biliary radicles
(black arrow). Inset shows a magnified view of the
(black arrow). Inset shows a magnified view of the
involved CBD (arrows).
involved CBD (arrows).

CT appearances of cholangiocarcinoma. (b)
CT appearances of cholangiocarcinoma. (b)
Multidetector CT scan through the liver with a
Multidetector CT scan through the liver with a
Bismuth-Corlette type IIIB cholangiocarcinoma
Bismuth-Corlette type IIIB cholangiocarcinoma
shows dilatation and crowding of left lobe biliary
shows dilatation and crowding of left lobe biliary
radicles (arrows) with an abrupt transition at the
radicles (arrows) with an abrupt transition at the
hilum (arrowhead) but no obvious mass. Type
hilum (arrowhead) but no obvious mass. Type
IIIB cholangiocarcinoma was confirmed with
IIIB cholangiocarcinoma was confirmed with
endoscopic retrograde cholangiography (ERC) and
endoscopic retrograde cholangiography (ERC) and
biopsy.
biopsy.

CT appearances of cholangiocarcinoma. (c)
CT appearances of cholangiocarcinoma. (c)
Multidetector CT scan obtained shows a
Multidetector CT scan obtained shows a
peripheral mass-forming lesion with delayed
peripheral mass-forming lesion with delayed
enhancement (arrowheads), ductal dilatation, and
enhancement (arrowheads), ductal dilatation, and
retraction of the liver surface. The CT appearances
retraction of the liver surface. The CT appearances
of cholangiocarcinomas vary depending on the
of cholangiocarcinomas vary depending on the
anatomic location of the lesion relative to the
anatomic location of the lesion relative to the
biliary tree.
biliary tree.

Hepatic tuberculosis. Contrast-
Hepatic tuberculosis. Contrast-
enhanced arterial phase CT scan
enhanced arterial phase CT scan
shows four layers of hepatic
shows four layers of hepatic
tuberculosis. Microscopic
tuberculosis. Microscopic
examination showed the
examination showed the
outermost (hyperattenuating) layer
outermost (hyperattenuating) layer
(arrowheads) to consist of
(arrowheads) to consist of
compressed normal hepatic
compressed normal hepatic
parenchyma with sinusoidal
parenchyma with sinusoidal
dilatation, the second
dilatation, the second
(hypoattenuating) layer (large
(hypoattenuating) layer (large
arrows) to consist mainly of
arrows) to consist mainly of
fibrosis, the third
fibrosis, the third
(hyperattenuating) layer (small
(hyperattenuating) layer (small
arrows) to represent
arrows) to represent
granulomatous inflammation, and
granulomatous inflammation, and
the innermost (hypoattenuating)
the innermost (hypoattenuating)
layer (
layer (*
*) to represent caseous
) to represent caseous
necrosis.
necrosis.

Periductal infiltrating cholangiocarcinoma.
Periductal infiltrating cholangiocarcinoma.
(a)
(a) Axial T2-weighted MR image shows a
Axial T2-weighted MR image shows a
dilated peripheral intrahepatic duct with
dilated peripheral intrahepatic duct with
a slightlyhyperintense lesion around the duct (arrow).
a slightlyhyperintense lesion around the duct (arrow).
(b)
(b) Contrast-enhanced MR image shows periductal
Contrast-enhanced MR image shows periductal
enhancement around the dilated intrahepatic duct
enhancement around the dilated intrahepatic duct
(arrowheads).
(arrowheads).
(c)
(c) Photograph of the gross specimen reveals a
Photograph of the gross specimen reveals a
periductal infiltrating tumor (arrows) along the
periductal infiltrating tumor (arrows) along the
irregularly dilated intrahepatic duct
irregularly dilated intrahepatic duct.
.

Cholangiocarcinoma
Cholangiocarcinoma
CT appearances of cholangiocarcinoma. (a) Coronal CT
image shows an infiltrating intraductal extrahepatic
cholangiocarcinoma causing irregular thickening and
enhancement (white arrows) of the CBD with dilatation of
intrahepatic biliary radicles (black arrow). (b) Multidetector
CT scan shows Bismuth-Corlette type IIIB
cholangiocarcinoma shows dilatation and crowding of left
lobe biliary radicles (arrows) with an abrupt transition at
the hilum (arrowhead) but no obvious mass. (c)
Multidetector CT scan shows a peripheral mass-forming
lesion with delayed enhancement (arrowheads), ductal
dilatation, and retraction of the liver surface. The CT
appearances of cholangiocarcinomas vary depending on
the anatomic location of the lesion relative to the biliary

Hepatic Cholangiocarcinoma
Hepatic Cholangiocarcinoma
(a) Noncontrast multidetector
CT scan shows an irregular hypoattenuating lesion in
segments VII and VIII of the liver (arrows) with
retraction of the posterior liver surface and atrophy of
the right lobe. (b) On an arterial phase multidetector
CT scan, the lesion is predominantly hypoattenuating
with minimal peripheral enhancement posteriorly
(arrow). (c) Delayed phase multidetector CT scan
shows the lesion with peripheral enhancement black
arrows) and progressive enhancement posteriorly
(white arrow), although the center of the lesion
remains hypoattenuating (*).

Klatskin tumour
Klatskin tumour
Klatskin tumor
(a) Multidetector CT scan shows dilatation of the intrahepatic biliary radicles
in both hepatic lobes (black arrows) with an abrupt cutoff at the hilum (white
arrow) but no discernible mass. (b) Corresponding coronal
minimumintensity- projection image shows the dilated biliary tree and
obstruction by a hilar cholangiocarcinoma (arrow).

Left lobe hepatic cholangiocarcinoma
Left lobe hepatic cholangiocarcinoma
Contrast-enhanced multidetector CT scans through the liver show a
heterogeneously enhancing mass in the left hepatic lobe (arrowheads
in a) causing segmental biliary dilatation and atrophy of the left lobe,
in conjunction with an enlarged lymph node in the gastrohepatic
space (arrow in a) and peritoneal metastases (arrow in b).

 Periductal infiltrating hilar cholangiocarcinoma.
Periductal infiltrating hilar cholangiocarcinoma. (a)
(a) Coronal T2-
Coronal T2-
weighted MR image shows irregularductal wall thickening along a
weighted MR image shows irregularductal wall thickening along a
narrowed hilar bile duct (arrow).
narrowed hilar bile duct (arrow). (b)
(b) Photograph of the gross
Photograph of the gross
specimen reveals an elongated and branchlike tumor along the
specimen reveals an elongated and branchlike tumor along the
bile duct.
bile duct.

 BilIN-3.
BilIN-3. (a)
(a) Axial contrast-enhanced CT scan shows diffuse ductal dilatation in the left hepatic
Axial contrast-enhanced CT scan shows diffuse ductal dilatation in the left hepatic
lobe through the common bile duct, with no visible intraductal mass.
lobe through the common bile duct, with no visible intraductal mass.
(b)
(b) Photomicrograph (original magnification,40; H-E stain) shows micropapillary
Photomicrograph (original magnification,40; H-E stain) shows micropapillary
tumors throughout the bile ducts. These findings are compatiblewith the micropapillary
tumors throughout the bile ducts. These findings are compatiblewith the micropapillary
form of BilIN-3 (carcinoma in situ).
form of BilIN-3 (carcinoma in situ).

 Intraductal papillary cholangiocarcinoma.
Intraductal papillary cholangiocarcinoma. (a)
(a) MR
MR
cholangiopancreatogram shows an intraductal polypoid mass with
cholangiopancreatogram shows an intraductal polypoid mass with
localized ductal dilatation (arrow).
localized ductal dilatation (arrow). (b)
(b) Photomicrograph (original
Photomicrograph (original
magnification, ×1; H-E stain) shows an intraductal growth type mass
magnification, ×1; H-E stain) shows an intraductal growth type mass
with focal ductal dilatation (arrowheads).
with focal ductal dilatation (arrowheads).

Cholangiocarcinoma
Cholangiocarcinoma
 A slow growing malignancy of the biliary tract
A slow growing malignancy of the biliary tract
which tend to infiltrate locally and metastasize
which tend to infiltrate locally and metastasize
late.
late.
 Gall Bladder cancer = 6,900/yr
Gall Bladder cancer = 6,900/yr
 Bile duct cancer = 3,000/yr
Bile duct cancer = 3,000/yr
 Hepatocellular Ca = 15,000/yr
Hepatocellular Ca = 15,000/yr

Cholangiocarcinoma
Cholangiocarcinoma
 90% are extra-hepatic
90% are extra-hepatic
 M = F
M = F
 60’s and 70’s
60’s and 70’s
 Highest incidence in Japan, Israel, and Native Americans
Highest incidence in Japan, Israel, and Native Americans
 Increased 3 fold in the last 30yrs in the USA
Increased 3 fold in the last 30yrs in the USA
 M/F=3/2
M/F=3/2

EPIDEMIOLOGY
Relatively uncommon malignancy
More common outside the United States,
particularly in South America and Eastern/Central
Europe
Less common than gallbladder cancer
Incidence per 100,000 in U.S.: 1.0 in females
1.5 in males
Increasing incidence with age
70% of cases in over 65 years
Hilar location most common
Cholangiocarcinoma

Epidemiology
Epidemiology
 Tumours of bile duct are rare-2% of all cancers found at autopsy.
Tumours of bile duct are rare-2% of all cancers found at autopsy.
 Malignant tumours more common than benign adenomas and
Malignant tumours more common than benign adenomas and
papillomas.
papillomas.
 Cholangiocarcinoma most common malignancy of bile ducts, >50%-
Cholangiocarcinoma most common malignancy of bile ducts, >50%-
Holland et at…2007.
Holland et at…2007.
 More common in Israel, Japan and American indians
More common in Israel, Japan and American indians
 Annual incidence of bile duct Ca in USA is 1/100,000 people. Autopsy
Annual incidence of bile duct Ca in USA is 1/100,000 people. Autopsy
studies show and incidence of 0.01-0.46%. 4,000 new cases reported
studies show and incidence of 0.01-0.46%. 4,000 new cases reported
annually in USA.
annually in USA.
 England and Wales - 2.8/100,000 females & 2/100,000 males.
England and Wales - 2.8/100,000 females & 2/100,000 males.

ETIOLOGY
ETIOLOGY
 Risk factors for bile duct cancer include:
Risk factors for bile duct cancer include:
 Ulcerative colitis
Ulcerative colitis
 Primary sclerosing cholangitis-10-30%
Primary sclerosing cholangitis-10-30%
 Parasitic infestations:Liver fluke common in Far East-
Parasitic infestations:Liver fluke common in Far East-
intrahepatic CC accounts for 20% of primary liver tumour.
intrahepatic CC accounts for 20% of primary liver tumour.
 Opisthorchis viverrini-found inThailand, and West
Opisthorchis viverrini-found inThailand, and West
Malaysia.
Malaysia.

ETIOLOGY
ETIOLOGY
 Toxic chemicals-thorium dioxide (thorotrast), radionuclides,
Toxic chemicals-thorium dioxide (thorotrast), radionuclides,
carcinogens-arsenic, nitrosamines
carcinogens-arsenic, nitrosamines
 Congenital fibrosis or cysts-cogenital hepatic fibrosis, cystic
Congenital fibrosis or cysts-cogenital hepatic fibrosis, cystic
dilatation, choledochal cyst, polycystic liver
dilatation, choledochal cyst, polycystic liver
 Drugs: methyldopa, isoniazide, OCP.
Drugs: methyldopa, isoniazide, OCP.
 Gallstones and hepatolithiasis-decrease incidence >10 years
Gallstones and hepatolithiasis-decrease incidence >10 years
post cholecystectomy.
post cholecystectomy.
 Biliary cirrhosis and typhoid carriers.
Biliary cirrhosis and typhoid carriers.

Cholangiocarcinoma
Cholangiocarcinoma
Etiology
Etiology
Ulcerative Colitis Thorotrast Exposure
Sclerosing Cholangitis Typhoid Carrier
Choledochal Cysts
Adult Polycystic
Kidney Disease
Hepatolithiasis
Liver Flukes
Papillomatosis of Bile
Ducts

Cholangiocarcinoma
Cholangiocarcinoma
Extra-hepatic: Distribution
Extra-hepatic: Distribution
 Right or left hepatic duct = 10%
Right or left hepatic duct = 10%
 Bifurcation = 20%
Bifurcation = 20%
 Proximal CBD = 30%
Proximal CBD = 30%
 Distal CBD = 30%
Distal CBD = 30%

Cholangiocarcinoma
Cholangiocarcinoma
Pathology
Pathology
 Almost all are adenocarcinoma
Almost all are adenocarcinoma
 Papillary, nodular, and sclerosing
Papillary, nodular, and sclerosing
 Best prognosis is with papillary
Best prognosis is with papillary
distal tumors
distal tumors

Exta-
Exta-
hepatic
hepatic
Bile
Bile
Duct
Duct
 Staging
Staging

PATHOPHYSIOLOGY
PATHOPHYSIOLOGY
 Bile duct tumours cause bile duct obstruction - biliary
Bile duct tumours cause bile duct obstruction - biliary
stasis and alteration of liver function tests
stasis and alteration of liver function tests
 Prolonged obstruction then leads to-
Prolonged obstruction then leads to-
 Hepatocellular dysfunction, renal dysfunction
Hepatocellular dysfunction, renal dysfunction
 Progressive malnutrition, Pruritus, coagulopathy
Progressive malnutrition, Pruritus, coagulopathy
 Cholangitis- esp if previous endoscopic, percutaneous or
Cholangitis- esp if previous endoscopic, percutaneous or
surgical biliary interventions have been performed.
surgical biliary interventions have been performed.

Anatomically, biliary tree is divided into 3 parts,
Anatomically, biliary tree is divided into 3 parts,
upper 3
upper 3rd
rd
-55%, middle 3
-55%, middle 3rd
rd
15% and lower 3
15% and lower 3rd
rd
10%.Of
10%.Of
these tumours, 10% are diffuse.
these tumours, 10% are diffuse.

Intra and Extra-hepatic Cholangiocarcinoma

Location
Location
Peripheral
• 7-20%
• Intrahepatic mass
• Cirrhosis uncommon
• Etiology unknown
Hilar
• 40-60%
• Biliary confluence
• Most common
Distal
• 20-30%
• 10-15% of
peripancreatic
tumors
Cholangiocarcinoma

Intraheptic Cholangio
Intraheptic Cholangio

Hilar Cholangiocarcinoma

De Oliviera et al. Ann Surg (2007)
Cholangiocarcinoma
Johns Hopkins Experience (1973-2004)
Intrahepatic
8%
Distal
42%
Perihilar
50%
12%
25%
38%

PREOPERATIVE EVALUATION
1. Cholangiography
• Assessment of extent of biliary ductal involvement
• ERCP vs MRCP vs PTC
2. Cross-sectional imaging
• Soft tissue extent, lobar atrophy, vascular
involvement, remnant volume, metastases
• CT vs MRI
Controversies:
• Role of preoperative stenting
• FDG-PET
• Staging laparoscopy

Cholangiocarcinoma
Cholangiocarcinoma
Diagnosis and Initial Workup
Diagnosis and Initial Workup
 Jaundice
Jaundice
 Wt loss, anorexia, abdominal pain, fever
Wt loss, anorexia, abdominal pain, fever
 US then CT (CTA?) Followed by ERCP, PTC
US then CT (CTA?) Followed by ERCP, PTC
or MRCP
or MRCP
 CEA and CA 19-9 can be elevated
CEA and CA 19-9 can be elevated

USS- Gallstone, grossly distended GB,
USS- Gallstone, grossly distended GB,
Markedly dilated CBD and IHBD.
Markedly dilated CBD and IHBD.

Non-Invasive Imaging: MRI/MRCP
• High quality images of the biliary tree - as good as
cholangioscopy for assessing biliary tumor extent*.
• Provides additional data regarding metastases, vascular
involvement, lobar atrophy.
Masselli et al Eu J Radiol (2008)
*Lee et al. Gastrointest Endosc 2002;56:25

CT- abdomen- intra & extra-hepatic bile duct
CT- abdomen- intra & extra-hepatic bile duct
dilatation to the level of the hepatic hilium.
dilatation to the level of the hepatic hilium.
Suggestion of 2cm mass at hilium-?
Suggestion of 2cm mass at hilium-?
cholangiocarcinoma-(Klatskin tumour)
cholangiocarcinoma-(Klatskin tumour)

CT appearances of cholangiocarcinoma.
(a) cholangiocarcinoma causing irregular
cholangiocarcinoma causing irregular
thickening and enhancementwhite arrows) of the CBD with upstream dilatation of
thickening and enhancementwhite arrows) of the CBD with upstream dilatation of
intrahepatic biliary radicles (black arrow). Inset showsa magnified view of the involved
intrahepatic biliary radicles (black arrow). Inset showsa magnified view of the involved
CBD (arrows).
CBD (arrows). (b)
(b) cholangiocarcinoma shows dilatation and crowding of left lobe biliary
cholangiocarcinoma shows dilatation and crowding of left lobe biliary
radicles (arrows) with an abrupt transition at the hilum
radicles (arrows) with an abrupt transition at the hilum (arrowhead) .
(arrowhead) .

Peripheral mass-forming cholangiocarcinoma (a) Noncontrast
CT scan
shows an irregular hypoattenuating lesion in segments VII
and VIII of the liver (arrows) with retraction of the posterior
liver surface and atrophy of the right lobe. (b) On an
arterial phase multidetector CT scan, the lesion is predominantly
hypoattenuating with minimal peripheral enhancement
posteriorly (arrow). (c) Delayed phase multidetector
CT scan shows the lesion with peripheral enhancement
(black arrows) and progressive enhancement posteriorly
(white arrow), although the center of the lesion remains
hypoattenuating (*).

MRCP-grossly dilated CBD/IHBD, abrupt
MRCP-grossly dilated CBD/IHBD, abrupt
narrowing of CBD with no obvious filling defect ?
narrowing of CBD with no obvious filling defect ?
cholangiocarcinoma. Grossly distended GB.
cholangiocarcinoma. Grossly distended GB.

MRCP of Extra-hepatic Cholangiocarcinoma at the Bifurcation
Klatskin tumor

Intrabiliary MRI
Arepally et al. (JHH)

ERCP + stenting the
ERCP + stenting the
left duct
left duct
ERCP :Kl
ERCP :Kl
atskin
atskin
tumor
tumor

Comparison of CT and ERCP
Comparison of CT and ERCP

Cholangiocarcinoma
i. (a) Noncontrast multidetectorCT scan obtain shows
an irregular hypoattenuating lesion in segments VIIand
VIII of the liver (arrows) with retraction of the
posteriorliver surface and atrophy of the right lobe. (b)
On anarterial phase multidetector CT scan, the lesion
is attenuating with minimal peripheral enhancement
posteriorly (arrow). (c) shows the lesion with
peripheral enhancement(black arrows) and progressive
enhancement posteriorly
(white arrow), although the center of the lesion
remainshypoattenuating (*).

• Most useful to rule out
metastatic disease.
• Less helpful for
cholangiocarcinoma than
for GB cancer.
• Consider in locally
advanced cases.
LAPAROSCOPIC STAGING

Role of FDG-PET
Anderson et al. J Gastrointest Surg 8:90 (2004)
• Not useful for infiltrating cholangiocarcinoma
• False negatives due to low volume metastases
• False positives due to stents or recent cholecystectomy

Bismuth Classification
Bismuth Classification
 Type i-involvement of common hepatic duct.
Type i-involvement of common hepatic duct.
 Type ii-bifurcation involved without involvement of
Type ii-bifurcation involved without involvement of
secondary intrahepatic duct.
secondary intrahepatic duct.
 Type iiia-extends into the right secondary intrahepatic
Type iiia-extends into the right secondary intrahepatic
duct.
duct.
 Type iiib-extends into the left secondary intrahepatic duct.
Type iiib-extends into the left secondary intrahepatic duct.
 Type iv- secondary intrahepatic ducts involved on both
Type iv- secondary intrahepatic ducts involved on both
sides.
sides.

Bismuth-Corlette classification scheme
for cholangiocarcinomas. MR
different patients. Drawings of the
lesions are shown in the corresponding
insets (cf Fig 2). Type I involves the CHD
with abrupt cutoff of the RHD and LHD
at the confluence (arrow in a); type II,
the CHD (thick arrow in b) and the
junction of the RHD and LHD (thin arrow
in b); type IIIA, the CHD (arrow in c),
biliary junction, and RHD (arrowheads in
c); type IIIB, the CHD (arrow in d), biliary
junction, and LHD (arrowhead in d); and
type IV, the CHD (arrow in e) and the
the RHD and LHD (arrowheads in e) or a
multifocal bile duct tumor. The Bismuth-
Corlette classification scheme can help
in planning the approach to and
management of cholangiocarcinomas.

Mass-forming peripheral cholangiocarcinoma in a 72-year-old woman.
Mass-forming peripheral cholangiocarcinoma in a 72-year-old woman. (a)
(a) Fat-
Fat-
saturated T1-weighted MR image obtained after the intravenous administration
saturated T1-weighted MR image obtained after the intravenous administration
of gadolinium-based contrast material shows a heterogeneously enhancing lesion
of gadolinium-based contrast material shows a heterogeneously enhancing lesion
(arrows).
(arrows). (b)
(b) On a fat-saturated T1-weighted MR image obtained after the
On a fat-saturated T1-weighted MR image obtained after the
administration of manganese dipyridoxylethylenediamine diacetate bisphosphate,
administration of manganese dipyridoxylethylenediamine diacetate bisphosphate,
the lesion (arrows) appears hypointense relative to the enhancing liver. The use
the lesion (arrows) appears hypointense relative to the enhancing liver. The use
of this contrast agent increases lesion-liver contrast and lesion conspicuity.
of this contrast agent increases lesion-liver contrast and lesion conspicuity.

 Intraductal infiltrating cholangiocarcinoma of the proximal CBD
Intraductal infiltrating cholangiocarcinoma of the proximal CBD
(a)
(a) Delayed phase contrast-enhanced fat-saturated T1-weighted
Delayed phase contrast-enhanced fat-saturated T1-weighted
MR image shows enhancement of the cholangiocarcinoma
MR image shows enhancement of the cholangiocarcinoma
(arrow).
(arrow). (b)
(b) On a diffusion-weighted image obtained at the same
On a diffusion-weighted image obtained at the same
level, the lesion (arrow) is hyperintense with restricted diffusion.
level, the lesion (arrow) is hyperintense with restricted diffusion.

PRESENTATION
PRESENTATION
 CC seen in advanced unresectable stage
CC seen in advanced unresectable stage
 Early diagnosis unusual
Early diagnosis unusual
 Typically elderly- average age 60-65years though Klatskin
Typically elderly- average age 60-65years though Klatskin
slightly younger age group
slightly younger age group
 Abnormal LFTs / Jaundice-90%
Abnormal LFTs / Jaundice-90%
 Abdominal pain / Weight loss- in (30-50%) of cases -Patel et
Abdominal pain / Weight loss- in (30-50%) of cases -Patel et
al 2006
al 2006
 Pruritus seen in 66% of patients
Pruritus seen in 66% of patients

PRESENTATION
PRESENTATION
 Fever- 20%
Fever- 20%
 Diarrhoea, anorexia, changes in urine & stool
Diarrhoea, anorexia, changes in urine & stool
colour and weight loss.
colour and weight loss.
 Liver may be enlarged and smooth-25-40%
Liver may be enlarged and smooth-25-40%
 Distended and non tender gallbladder 10%
Distended and non tender gallbladder 10%
 Epigastric tenderness.
Epigastric tenderness.

DIAGNOSIS
DIAGNOSIS
 History / physical examination
History / physical examination
 Labouratory-CEA and CA19.9 –sensitivity of 66% and a
Labouratory-CEA and CA19.9 –sensitivity of 66% and a
specificity of 100% in diagnosing CC in pt with PSC.
specificity of 100% in diagnosing CC in pt with PSC.
 Imaging-tumours are generally small-USS/ CT may fail to
Imaging-tumours are generally small-USS/ CT may fail to
show the lesion.
show the lesion.
 Cholangiography via a transhepatic or endoscopic
Cholangiography via a transhepatic or endoscopic
approach reqired to define biliary anatomy and extent of
approach reqired to define biliary anatomy and extent of
the lesion.
the lesion.

• Complete resection is the only effective therapy.
• Outcomes after R0 resection:
– 5-year overall survival of 25-40%
– DFS of 15-25%
• Few patients are resectable.
• R1/2 resections are not uncommon.
• Palliating the effects of biliary obstruction is often the
primary treatment objective.
Treatment

Cholangiocarcinoma
Cholangiocarcinoma
Intra-hepatic Disease-Surgery/Ablation
Intra-hepatic Disease-Surgery/Ablation
 Extent of surgical therapy is determined by the
Extent of surgical therapy is determined by the
location, hepatic function, and underlying cirrhosis.
location, hepatic function, and underlying cirrhosis.
 Anatomic resections have lowest recurrence rates.
Anatomic resections have lowest recurrence rates.
However nonanatomic resection increases
However nonanatomic resection increases
potential surgical candidates and improves survival.
potential surgical candidates and improves survival.
 Hepatic devascularization prior to resection is
Hepatic devascularization prior to resection is
preferred
preferred
 Ablative therapy gives good local control
Ablative therapy gives good local control.
.

Child’s Classification
Child’s Classification
Class Alb Bili Ascites
Malnutri-
tion
Encephal-
opathy
Surgical
Mortality
A >3.5 <2.0 0 0 0 5%
B 3-3.5 2-3 Controlled Mild Minimal 10-20%
C <3 >3
Poor
Control
Significant
Recurrent/
Persistent
30-40%

Cholangiocarcinoma
Cholangiocarcinoma
Intra-hepatic Disease: Extent of Resection
Intra-hepatic Disease: Extent of Resection
 No Cirrhosis: 60% of liver
No Cirrhosis: 60% of liver
 Mild Cirrhosis with normal LFT’s: one lobe,
Mild Cirrhosis with normal LFT’s: one lobe,
maybe
maybe
 Moderate Cirrhosis with mild LFT abnormality
Moderate Cirrhosis with mild LFT abnormality
(Child’s B): Wedge resection/RFA
(Child’s B): Wedge resection/RFA
 Child’s C: no surgical therapy
Child’s C: no surgical therapy

Cholangiocarcinoma
Cholangiocarcinoma
Intra-hepatic Disease
Intra-hepatic Disease
 Locally aggressive tumor: 65% present with
Locally aggressive tumor: 65% present with
satellite nodules, perineural invasion
satellite nodules, perineural invasion
 For residual disease use Radiation therapy and 5-
For residual disease use Radiation therapy and 5-
FU based therapy or gemcitabine
FU based therapy or gemcitabine
 Re-image all every 6 mo for 2 yr. Start workup
Re-image all every 6 mo for 2 yr. Start workup
over for a new mass.
over for a new mass.

Cholangiocarcinoma
Cholangiocarcinoma
Extra-hepatic Disease: Surgical Therapy
Extra-hepatic Disease: Surgical Therapy
 CT +/- cholangiogram
CT +/- cholangiogram
 If proximal, resect back to secondary bifurcation
If proximal, resect back to secondary bifurcation
or one lobe and primary bifurcation, take nodes
or one lobe and primary bifurcation, take nodes
and caudate lobe. Stent anastamoses.
and caudate lobe. Stent anastamoses.
 If Mid CBD, excise back to negative margins
If Mid CBD, excise back to negative margins
and create Roux en Y hepaticojejunostomy.
and create Roux en Y hepaticojejunostomy.
 For distal disease: Whipple
For distal disease: Whipple

Node Dissection in Bile Duct Excision

Cholangiocarcinoma
Cholangiocarcinoma
Extra-hepatic Disease: Unstentable
Extra-hepatic Disease: Unstentable
 Bypass if possible
Bypass if possible
 If not use proximal decompression and
If not use proximal decompression and
feeding jejunostomy
feeding jejunostomy
 Chemotherapy/Radiation Therapy/Brachy
Chemotherapy/Radiation Therapy/Brachy
therapy as tolerated or clinical trial.
therapy as tolerated or clinical trial.

Patient-Related Factors
• Medical contraindication to major abdominal surgery
• Cirrhosis or insufficient remnant hepatic volume
Metastatic Disease
• N2 lymphadenopathy
• Distant metastases
CRITERIA OF UNRESECTABILITY

Local Tumor-Related Factors
• Tumor extension to secondary biliary radicles
bilaterally
• Encasement or occlusion of the main portal vein
proximal to its bifurcation
• Unilateral tumor extension to secondary bile ducts
with contralateral vascular encasement or occlusion
• Atrophy of one hepatic lobe with contralateral portal
vein encasement or secondary biliary extension
CRITERIA OF UNRESECTABILITY

Bismuth-Corlette Classification of Biliary Extent of

ESTABLISHED:
• Excision of supraduodenal bile duct
• Cholecystectomy
• Restore bilioenteric continuity
Goal of Resection:
Complete Tumor Excision with Negative Margins
LESS CONTROVERSIAL:
• Routine hepatectomy/caudate (left resections)
• Portal lymphadenectomy
• Selected major vascular reconstruction
MORE CONTROVERSIAL:
• Routine PV resection (Neuhaus)
Recommended

Biliary-enteric Anastomosis
Biliary-enteric Anastomosis

Biliary Stents for the Management of Surgically
Biliary Stents for the Management of Surgically
Unresectable Cholangiocarcinoma
Unresectable Cholangiocarcinoma

Management of Surgically Unresectable
Management of Surgically Unresectable
Cholangiocarcinoma
Cholangiocarcinoma
Percutaneous vs. Endoscopic Stenting?
• Superior technical and clinical success with endo
• Better control of bilirubin levels
• Significantly fewer complications
• Lower 30-day mortality rate
Speer et al. Lancet (Jul 1987)

Cholangiocarcinoma
Cholangiocarcinoma
Prognosis
Prognosis
 Best Result are with distal CBD tumors completely
Best Result are with distal CBD tumors completely
excised. Cure = 40%
excised. Cure = 40%
 Incomplete resection plus radiation gives a median
Incomplete resection plus radiation gives a median
survival of 30 m.
survival of 30 m.
 Stenting plus chemo/radiation gives a median
Stenting plus chemo/radiation gives a median
survival of 17 to 27m
survival of 17 to 27m
 Those stented alone live only a few months
Those stented alone live only a few months

1. Achieving complete margin negative resection remains
the goal in selected patients with hilar
cholangiocarcinoma, requiring hepatic resection in
nearly all cases.
2. Advances in non-invasive imaging have allowed better
identification of unresectable cases.
3. The role of PET, laparoscopic staging remain
controversial.
4. The choice of stent palliation approach (endo vs perc)
should individualized.
Summary
Surgical Management of Cholangiocarcinoma

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