This document discusses radiological imaging techniques for cholangiocarcinoma (bile duct cancer). It provides details on:
- Ultrasound, CT, MRI, MRCP, and ERCP are discussed for imaging bile duct cancer. Each modality has benefits for assessing tumor location and extent.
- Peripheral, hilar, and intrahepatic cholangiocarcinoma are described along with the Bismuth-Corlette classification system for hilar tumors.
- Imaging features of peripheral, hilar, intrahepatic cholangiocarcinoma are shown including enhancement patterns and involvement of bile ducts.
4. Biliary tract cancer is the second most common primary
hepatobiliary malignancy after hepatocellular carcinoma.
Malignancies may occur along any part of the biliary tract
from the ampulla of Vater to the smallest intrahepatic ductules
and the gallbladder. The entire biliary tree, including the
gallbladder is lined with a simple columnar epithelium and
malignant transformation of this epithelium gives rise to
predominantly adenocarcinomas. The pathogenesis of biliary
tract and gallbladder carcinoma is thought to be secondary to
an evolutionary sequence from metaplasia to dysplasia to
carcinoma. Metaplasia usually occurs in the setting of
inflammation and chronic injury. Dysplasia of the biliary tract,
is considered as pre-invasive biliary neoplasia and can occur in
up to 40% to 60% of patients with invasive carcinoma, one
third of patients with sclerosing cholangitis, and found
incidentally in 1 to 3.5% of cholecystectomy specimens
5. Classically, the cancers of the biliary tract were separated into
three categories: (i) cancer of the intrahepatic biliary tract, (ii)
cancer of the gallbladder and extrahepatic bile ducts, and (iii)
cancer of the ampulla of Vater. The term cholangiocarcinoma
was initially used to refer only to the primary tumors of the
intrahepatic bile ducts and is now extended to include
intrahepatic, perihilar, and distal extrahepatic tumors of the
bile ducts.
Gallbladder cancer is defined as cancer arising from the
gallbladder and the cystic duct. Ampullary cancers are rare
and have better prognosis than cancers of the distal bile duct.
Cancers arising from the distal common bile duct immediately
adjacent to the ampulla of Vater tend to behave clinically
similar to the cancers of the ampulla of Vater, head of pancreas
and the duodenal bulb and therefore often considered under
the broad category of periampullary carcinomas.
6. Cholangiocarcinoma: Pathogenesis and Epidemiology
Bile duct cancer arising in the intrahepatic,
peri-hilar, or distal biliary tree, exclusive of
gallbladder and ampulla of Vater.
Originate from epithelial cells of biliary duct
90% adenocarcinoma, 10% squamous cell
carcinoma.
Highly lethal: often locally advanced at
presentation.
5-year-survival rate: 5-10%.
7. Risk Factors
Primary sclerosing cholangitis
Life time risk: 10-15%
Average age at time of diagnosis: 30s-50s
Fibropolycystic liver disease
i.e. Caroli’s syndrome, congenital hepatic fibrosis, choledochal
cysts
Lifetime risk: 15%
Average age at time of diagnosis: 30s-50s
Parasitic Infection Liver flukes of Clonorchis and Opisthorchis
genera (from undercooked fish)
Cholelithiasis and haptolithiasis
Toxic exposure Thorotrast
Lynch syndrome and biliary papillomatosis
Chronic liver disease
Viral hepatitis.
8. Common Clinical Manifestations
Symptoms: Pruritus (66%)
RUQ pain (30-50%)
Weight loss (30-50%)
Fever (20%)
Alcoholic stools; dark
urine
Cholangitis(rare
Physical Signs: Jaundice
(90%)
Hepatomegally (25-40%)
RUQ mass (10%)
Courvoisier’s sign (rare)
9. Tumor Classification.
Intrahepatic(Peripheral): small intra hepatic ductules(5-10%)(yellow).
Hilar: extrahepatic ductules (including confluence) up to point where
common bile duct lies posterior to duodenum(60-70%)Klatskin:
involving confluence of left and right hepatic ducts. (blue).
Extrahepatic(Distal): originate in extrahepatic biliary duct after CBD
travels posterior to duodenum (20-30%).(Orange).
10. Drawings illustrate various types of intrahepatic
Cholangiocarcinoma as classified by the Liver Cancer
Study Group of Japan. (a) Mass-forming intrahepatic
Cholangiocarcinoma manifests as a round mass
with a distinct border in the liver parenchyma. (b)
Periductal infiltrating intrahepatic Cholangiocarcinoma is
characterized by tumor infiltration along the bile duct
(arrow). It occasionally involves the surrounding blood
vessels or hepatic parenchyma. (c) Intraductal
intrahepatic cholangiocarcinoma is characterized by
papillary or granular growth within the bile duct lumen.
It occasionally demonstrates superficial extension (right
arrow) or forms a tumor thrombus in an obstructed duct
(left arrow). More than one type of cholangiocarcinoma
may manifest in a single patient. In such cases, all of the
types involved should be
11. Bismuth-Corlette Classification
Hilar cholangiocarcinomas are
further sub-classified based on
the specific ducts involved Type I
Type II
Type IIIa
Type IIIb
Type IV
Type IV is important because it
involves both left and right
hepatic ducts and therefore is
unrespectable.
12. Schematic diagram of the
Bismuth–Corlette classification
scheme for perihilar tumors. Type
I: tumor confined to CHD duct,
obstructing the duct within 2 cm
of the hilum. Type II: tumor of the
CHD bifurcation involving both
main right and left hepatic ducts,
and causing obstruction at the
hilum with no communication
between the main right and left
hepatic ducts. Type IIIa and IIIb:
tumors extending into right and
left secondary intrahepatic ducts,
respectively, with absence of
ductal obstruction on the
contralateral side. Type IV: tumor
involves the secondary and
tertiary intrahepatic ducts in both
lobes causing bilateral
obstruction.
13. TNM Tumor Staging
Stage Grouping:
Stage I: T1 N0 M0
Stage II: T2 N0 M0
Stage IIIA: T3 N0 M0
Stage IIIB: T4 N0 M0
Stage IIIC: any T N1 M0
Stage IV: any T any N
T0 No evidence of primary tumor
T1 Solitary tumor w/o vascular
invasion.
T2 Solitary tumor with vascular
invasion, or multiple tumors <5cm.
T3 Multiple tumors >5cm or tumor
involving major branch of portal
or hepatic veins.
T4 Tumors with direct invasion of
adjacent organs other than the
gallbladder or with perforation
of the visceral peritoneum.
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
M0 No distant metastasis
M1 Distant metastasis
16. Peripheral cholangiocarcinoma. (a) Arterial-phase CT scan shows a low-attenuation
mass (marker) with rim enhancement. Note the dilatation of the peripheral
intrahepatic ducts (arrows). (b) On a portal-phase CT scan, the mass looks smaller
because the central portion is now more enhanced. The rim enhancement seen in a
is partially washed out. Capsular retraction is also noted (arrow).
17. Peripheral mass-forming cholangiocarcinoma
(a) Noncontrast multidetector CT scan obtained prior
to contrast material administration 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 (*).
18. Peripheral cholangiocarcinoma with involvement of confluence. Infiltrating pCCA of
the left hepatic duct (arrow) isodense to liver parenchyma on axial contrast enhanced
CT (a) with dilation of the left hepatic ducts. The ductal thickening is hyperintense
(arrow) on T2-weighted MRI image (b) with extension to the confluence causing mild
dilatation of the right hepatic ducts demonstrated better on MRCP (c).
19. peripheral cholangiocarcinoma. (a) Fat-saturated T1-weighted MR image obtained after
the intravenous administration of gadolinium-based contrast material shows a
heterogeneously enhancing lesion (arrows). (b) On a fat-saturated T1-weighted MR image
obtained after the administration of manganese dipyridoxylethylenediamine diacetate
bisphosphate, the lesion (arrows) appears hypointense relative to the enhancing liver. The
use of this contrast agent increases lesion-liver contrast and lesion conspicuity.
20. Peripheral cholangiocarcinoma demonstrates peripheral heterogeneous
enhancement. The tumor has been outlined to indicate a region of interest for the
calculation of vascularization parameters. The decrease in tumor vascularization
after antiangiogenic drug administration, seen in b, was confirmed with transfer
constant and extracellular volume fraction calculations.
21. Intrahepatic cholangiocarcinoma (iCCA) on ultrasound in two different
patients. Mass forming iCCA may present as a well-defined hypoechoic mass
(arrow, a) or as an ill-defined heterogeneous isoechoic mass (arrowheads, b).
22. Intraductal intrahepatic cholangiocarcinoma in a 53-year-old man. (a) CT scan shows a
dilated bile duct in segment VII of the liver (arrows). The bile duct has higher
attenuation in the right lobe than in the left lobe. (b) On a T2-weighted MR image, the
signal intensity of the right posterior superior intrahepatic duct (B7) (open arrows) is
not as high as that of other bile ducts (solid arrows). (c) Photograph of the resected
specimen shows granular, friable papillary masses (arrows) in dilated intrahepatic
ducts. (Fig 3a and 3c reprinted, with permission, from reference 11.)
23. CCA of common hepatic duct with involvement of confluence. Contrast enhanced
CT (a), T2-weighted MRI (b) and MRCP (c) images demonstrating thickened and
enhancing common hepatic duct (arrow) with involvement of the confluence and
upstream dilatation of the intrahepatic ducts. The ductal thickening appears
hypointense (arrow) to the surrounding dilated bile ducts on T2-weighted MRI
image (b). The involvement of the confluence is demonstrated better on MRCP.
24. CCA of common hepatic duct with involvement of confluence. Ultrasound with color flow overlay (a)
showing an echogenic mass (arrow) filling the common hepatic duct with upstream dilatation of the
intrahepatic ducts. EUS image (b) showing a hilar lymph node and was sampled positive for carcinoma.
Contrast enhanced CT in arterial phase (c) and portal venous phase (d) showing enhancing mass (arrow)
within the duct and no major vascular involvement. The ductal mass is hyperintense on T2-weighted
image (e) and hypointense on T1-weighted image (f) and shows concentric post contrast enhancement
(g). MRCP (h) shows the confluence invasion and non- visualization of the common hepatic duct.
25. MRCP of a classical Klatskin’s tumor. The confluence, proximal hepatic ducts and proximal
common hepatic duct are strictured (arrow). The common bile duct is of normal caliber.
26. Hilar CCA presenting as an intraluminal mass with biliary obstruction. The mass
is isodense (arrow) on the coronal non-contrast enhanced CT (a) and
hypointense on axial T2-weighted image (b) with upstream dilatation of the
intrahepatic ducts. ERCP (c) shows a filling defect (arrow) representing the mass
extending into the common bile duct with no filling of the intrahepatic ducts.
27. Perihilar CCA arising from the cystic duct and proximal gall bladder (arrows) with invasion
of common hepatic duct. The pCCA is mildly hyperintense on T2-weighted image (a),
hypointense on T1-weighted image (b) and shows post contrast enhancement (c). Both
MRCP (d) and ERCP (e) demonstrate the stricture of the common hepatic duct till the
confluence above and cystic duct insertion below. The gall bladder is not visualized on ERCP
and the irregularity of the proximal cystic duct (arrowhead) is due to the tumor.
28. Cholangiocarcinoma. (a) Axial fat-suppressed T2-weighted MR image shows a high-signal-
intensity lobulated mass in the right hepatic lobe (arrow). (b, c) Contrast-enhanced arterial
phase (b) and equilibrium phase (c) T1-weighted MR images show irregular, ragged rim
enhancement (arrows in b) with gradual centripetal enhancement (arrowheads in c).
29. Polypoid type iCCA. Axial T2 sections (a, b) and MRCP (c) demonstrating an isointense
filling defect (white arrow) in the right hepatic duct and extending into the common
hepatic duct with dilation of intrahepatic ducts. Post contrast enhanced T1-weighted
images (d-f) shows the mildly enhancing filling defect representing intraductal
papillary neoplasm which extended from just under hepatic capsule filling right
hepatic ducts to 3 cm below the confluence of right and left hepatic ducts.
30. Mixed type iCCA. T2-weighted axial (a), MRCP (b), T1-weighted axial (c) and post
contrast T1-weighted axial (d) images demonstrating a predominantly periductal
thickening (stricturing iCCA) and also mass forming (arrow) in the right lobe liver.
Note the separation of the right intrahepatic ducts on MRCP (arrowheads).
31. Cirrhosis of liver with iCCA. T2-weighted axial (a), T1-weighted axial (b) and post
gadolinium enhanced T1-weighted axial arterial phase (c), portal venous phase (d) and
delayed phase (e) images showing iCCA as an iso- to hyperintense lesion (arrow) in
posterior right lobe with typical arterial phase rim like enhancement and progressive
central enhancement through delayed phase without any washout. The liver
parenchyma is heterogeneous and nodular consistent with cirrhosis.
33. Intrahepatic cholangiocarcinoma in a 35-year-old female who presented with right upper quadrant abdominal pain and
no prior medical history. There is a large lobulated mass (arrow) in the right lobe of the liver, which, compared to liver
parenchyma, is hypointense on the (a) axial T1-weighted (T1W) in-phase spoiled gradient-echo (SGRE) image, and
mildly hyperintense on the (b) axial T2-weighted (T2W) fast spin echo (FSE) with fat saturation image. The mass shows
heterogeneous rim enhancement on the (c) arterial-dominant phase gadolinium-enhanced axial T1W 3D SGRE image
with fat saturation, and fills in heterogeneously on the (d) delayed (15 min) gadolinium-enhanced axial T1W SGRE
image. A presumed metastatic nodule (white open arrow) is seen in the lateral segment of the left lobe on an (e) axial
T2W FSE with fat suppression image. CT guided biopsy of the dominant mass revealed a cholangiocarcinoma.
34. Infiltrating hilar cholangiocarcinoma with tumoral involvement of the right secondary
confluence and common hepatic duct. (a) CT scan reveals a high-attenuation tumor on the
anterior aspect of the right portal vein (arrowheads). (b) On a subsequent CT scan, the tumor
appears as a high-attenuation lesion on the right side of the portal vein (arrowheads). (c, d)
CT scans (d obtained at a lower level than c) show the mucosa of the cystic duct with strong
enhancement (open arrow), a finding that suggests tumoral involvement. Soft tissue infiltration
around the portal vein (arrowheads) and lymphadenopathy (solid arrow) are also noted.
35. Perihilar cholangiocarcinoma (Klatskin
tumor) in a 62-year-old female
presenting with painless jaundice.
There is mass (arrow) at the junction
of the main right and left hepatic
ducts. The mass is seen on (a) an MRCP
image as a focal stricture involving the
duct bifurcation with dilatation of the
intrahepatic ducts, left more than
right, due to the presence of a right
intrabiliary stent. In (b) T1W in-phase
SGRE, the mass is hypointense relative
to liver parenchyma, and mildly
hyperintense on (c) T2W FSE with fat
suppression. A susceptibility artifact
from the right intrahepatic stent is
demonstrated (asterisk). The mass
enhances progressively (d–g) following
gadolinium administration: in (d) the
arterial-dominant phase, (e) portal-
venous phase, (f) interstitial phase (2
min post-gadolinium), and is best
depicted on (g) the 15 min post-
gadolinium image as a hyperintense
mass relative to adjacent liver
parenchyma.
36. Intraductal infiltrating cholangiocarcinoma of the proximal CBD. (a) Delayed phase
contrast-enhanced fat-saturated T1-weighted MR image shows enhancement of
the cholangiocarcinoma (arrow). (b) On a diffusion-weighted image obtained at the
same level, the lesion (arrow) is hyperintense with restricted diffusion.
37. Intraductal
cholangiocarcinoma in a
70-year-old male who
presented with jaundice.
An intraductal mass was
found on ERCP. There is a
minimally enhancing mass
within the right main
hepatic duct (arrow) on
the (a) axial gadolinium-
enhanced T1 SGRE image.
The mass extends to the
bifurcation of the ducts,
and has resulted in
intrahepatic ductal
dilatation. The intraductal
cholangiocarcinoma is
seen as a filling defect
(arrow) on the (b) coronal
T2W SSFSE MRCP images.
Cytology and brushings
revealed a
cholangiocarcinoma.
38. Hilar cholangiocarcinoma in a 62-year-old man who presented with jaundice and
abdominal pain. (a) Coronal fat-saturated T2-weighted MR image through the liver reveal
a mass at the hilum (arrow) with dilatation of the intrahepatic biliary radicals
(arrowheads). (b) FDG PET scan through the hilum (arrow) does not show increased
activity. The lesion proved to be a cholangiocarcinoma at histopathologic analysis.
40. Distal CCA. Common bile duct stricture due to grade 4 invasive carcinoma. Coronal T2-weighted
image (a) and MRCP (b) image showing a short segmental narrowing (arrow) with proximal
dilatation. ERCP (c) showing a short segmental stricture representing the invasive CCA.
41. Distal CCA presenting as a polypoid mass with obstructive jaundice. Contrast enhanced
CT axial (a) and coronal reconstruction (b) showing a soft tissue density filling defect in
distal common bile duct (arrow) representing the invasive grade 3 adenocarcinoma.
42. Polypoid extrahepatic cholangiocarcinoma with diffuse bile duct involvement in a 65-year-
old man. (a, b) CT scans (b obtained at a lower level than a) show a dilated common bile
duct filled with a papillary tumor (arrow). (c) CT scan shows partial restoration of the
ductal lumen in the intrapancreatic portion of the common bile duct. However, small
papillary tumors are still evident (arrow). (d) On a CT scan obtained at the level of the
distal common bile duct, the lumen is again filled with an intraductal papillary tumor
(arrow). (e) Direct cholangiogram shows a large papillary tumor in the proximal two-thirds
and the distal portion of the common bile duct (arrows). Because of this extensive tumoral
involvement, palliative resection was the only possible treatment.
43. Perihilar cholangiocarcinoma in a 64-year-
old female who presented with painless
jaundice and had focal stenosis of the
common bile duct on ERCP. There is a
perihilar mass (arrow), which is
hypointense to liver on (a) axial T1W in-
phase SGRE, mildly hyperintense on (b)
T2W FSE with fat saturation, has little to
no arterial enhancement on (c) the
arterial-dominant phase gadolinium-
enhanced axial T1W 3D SGRE, mild
enhancement on the (d) 2 min post-
gadolinium image, and the tumor becomes
mildly hyperintense to liver on the (e) 15
min post-gadolinium image. The tumor
surrounds and narrows the main portal
vein (white open arrow), and the right
main hepatic artery (black open arrow) on
a (f) higher slice. (g) Coronal thick slab
T2W single shot fast spin-echo (SSFSE)
MRCP image demonstrates long segment
narrowing of the common hepatic and
proximal common bile duct, with a stent
traversing the stenotic segment as well as
dilation of the intrahepatic bile ducts. (h) A
corresponding minimum intensity
projection (Min IP) reformatted image
demonstrates the mildly enhancing mass
surrounding the narrowed segment of
common hepatic duct (curved arrow).
44. Cholangiocarcinoma. (a) Out-of-phase gradient-echo T1-weighted MR image shows a
hypointense lobulated mass in the right hepatic lobe (arrows). (b) On a fat-saturated
T2-weighted MR image, the mass appears hyperintense (arrows). (c) Early-phase
contrast-enhanced T1-weighted MR image shows irregular peripheral enhancement of
the mass (arrows). (d) Delayed phase contrast-enhanced T1-weighted MR image
shows progressive heterogeneous enhancement of the lesion (*).
45. Cholangiocarcinoma. (a) 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 radicals in both
lobes (arrowheads). (b) Out-of-phase gradient echo T1-weighted MR image shows the mass encasing
the left portal vein (arrow) and extending to the porta hepatis. (c, d) Corresponding contrast-enhanced
MR angiograms show encasement of the left hepatic artery (arrow in c) and invasion and non
visualization of the left portal vein (arrow in d), which prohibited surgery.
47. PET-CT of iCCA. Axial PET-CT images showing a large FDG-avid iCCA (arrow) with central
photopenia (*) indicating necrosis/fibrosis with FDG-avid portal lymph node (a, arrow
head) and aortocaval lymph node (b, arrow head) consistent with lymph node metastases.
48. Ampullary carcinoma. Contrast enhanced axial CT (a) and coronal reformat (b) showing a
small polypoid mass (arrow) representing carcinoma in the ampulla of the bile duct.
49. Ampullary carcinoma. Contrast enhanced axial CT (a) and sagittal reformat (b) showing
a small polypoid mass representing carcinoma in the ampulla of the bile duct.
54. Summary.
Radiographic diagnosis, and an even more
challenging cancer to treat.
3 classifications of Cholangiocarcinoma:
Intrahepatic
Hilar(including Klatskin)
Extrahepatic
Hilar cholangiocarcinomas are further sub-classified based on
specific ductal involvement, by the Bismuth-Corlette classification
system.
Many radiographic modalities are important in the diagnosis of
Cholangiocarcinoma.
US, CT, ERCP, MRCP, MRI
Radiology is helpful in determining surgical respectability, and can
influence surgical management.