This document provides information about cholangiocarcinoma, a malignant tumor arising from the biliary tree. It discusses the incidence, clinical presentation, locations, growth patterns, staging, and radiographic features. Cholangiocarcinoma is usually seen in the elderly and presents with painless jaundice. It can be located in the hilar region or peripherally. On imaging, it may appear as a mass, infiltrate along bile ducts, or have an intraductal growth pattern. Staging uses the Bismuth-Corlette classification. Key radiographic findings include dilated intrahepatic ducts, hilar lesions causing central obstruction without a clear mass, and encasement of portal veins

1. Gastro-Intestinal Tract
Cholangiocarcinoma

2. Mohamed Zaitoun
Assistant Lecturer-Diagnostic Radiology
Department , Zagazig University Hospitals
Egypt
FINR (Fellowship of Interventional
Neuroradiology)-Switzerland
zaitoun82@gmail.com

5. Knowing as much as
possible about your enemy
precedes successful battle
and learning about the
disease process precedes
successful management

6. Cholangiocarcinoma
1-Incidence
2-Clinical Picture
3-Location
4-Growth Patterns
5-Staging
6-Radiographic Features

7. 1-Incidence :
-Is a malignant tumor arising from the biliary
tree and tends to have a poor prognosis
and high morbidity
-It is the second most common primary
hepatic tumor with intra-hepatic
cholangiocarcinoma accounting for 10-
20% of primary liver tumors
-Usually in the elderly (7th decade)

8. 2-Clinical Picture :
-Painless jaundice
3-Location :
a) Perihilar : originates from epithelium of main
hepatic ducts or junction : Klatskin tumor
b) Peripheral : originates from epithelium of
intralobular ducts (beyond second-order bile
ducts)

9. 63-year-old man with hilar cholangiocarcinoma, intercostal sonographic
scan through common hepatic duct shows well-defined soft-tissue
intraductal mass (white arrow) within dilated intrahepatic duct (black
arrow), S8 = segment VIII

10. 58-year-old man with hilar cholangiocarcinoma, subcostal oblique gray-
scale sonographic scan through porta hepatis shows abrupt
narrowing of right intrahepatic duct (black arrows) secondary to
infiltrating tumor (white arrow)

11. 58-year-old man with hilar cholangiocarcinoma. Contrast-enhanced CT
scan shows tumor (white arrows) infiltrating right portal vein (black
arrow)

12. Klatskin tumor in a 57-year-old man who presented with jaundice, weight loss,
and abdominal pain, (a) CT scan shows dilatation of the intrahepatic biliary
radicals in both hepatic lobes (black arrows) with an abrupt cutoff at the
hilum (white arrow) but no discernible mass, (b) Corresponding coronal
minimum-intensity-projection image shows the dilated biliary tree and
obstruction by a hilar cholangiocarcinoma (arrow)

13. Peripheral mass-forming cholangiocarcinoma in a 73-year-old woman, (a) Noncontrast
CT 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, the lesion is predominantly hypoattenuating with
minimal peripheral enhancement posteriorly (arrow), (c) Delayed phase shows the
lesion with peripheral enhancement (black arrows) and progressive enhancement
posteriorly (white arrow), although the center of the lesion remains hypoattenuating
(*)

14. 4-Growth Patterns :
-Cholangiocarcinoma is classified into :
a) Mass-forming
b) Periductal infiltrating
c) Intraductal growth

16. a) Mass-forming :
-Mass-forming cholangiocarcinoma is
characterized morphologically by a
homogeneous mass with an irregular but well-
defined margin and is frequently associated with
dilatation of the biliary trees in the tumor
periphery
-Vascular encasement by the tumor is also
common, but grossly visible intravascular tumor
thrombosis is rare

17. Mass-forming intrahepatic cholangiocarcinoma manifests as a round
mass with a distinct border in the liver parenchyma

19. *US :
-Manifests as a homogeneous mass with an
irregular but well-defined margin
-A peripheral hypoechoic rim is seen in about 35%
of all tumors and consists of compressed liver
parenchyma or proliferating tumor cells
-Tumors greater than 3 cm in size are usually
hyperechoic, but tumors less than 3 cm are
hypo- or isoechoic

20. Mass-forming peripheral cholangiocarcinomas, (a) Transabdominal US image
obtained in a 73-year-old woman shows a hypoechoic lesion (arrows),
(b) Transabdominal US image obtained in a 41-year-old man shows a
hyperechoic lesion (arrows), (c) Transabdominal US image obtained in a
66-year-old woman shows a mixed-echogenicity lesion (arrowheads) with
biliary dilatation (arrow)

21. *CT :
-Homogeneous attenuation, irregular peripheral
enhancement with gradual centripetal
enhancement, capsular retraction, the presence
of satellite nodules, and vascular encasement
without the formation of a grossly visible tumor
thrombus
-Other common findings include the presence of
hepatolithiasis associated with the ductal
dilatation and obliteration of the portal vein,
leading to atrophy of the involved segment

22. Typical features of mass-forming cholangiocarcinoma at CT, (a) Arterial phase CT scan
shows a tumor with ragged rim enhancement at the periphery (arrow), (b) Axial portal
venous phase CT scan shows gradual centripetal enhancement of the tumor with
capsular retraction (black arrow), a satellite nodule is also seen (white arrow), (c)
Three-minute delayed phase CT scan shows gradual centripetal enhancement with
tumor encasement of the posterior branch of the right portal vein (arrowhead),
encasement of a portal or hepatic vein without formation of a grossly visible tumor
thrombus is one of the distinguishing features of cholangiocarcinoma as opposed to
HCC

23. 58-year-old woman with mass-forming intrahepatic
cholangiocarcinoma, CT scan shows lobulated tumor with sharp but
irregularly rolled margin in right hepatic lobe

24. 57-year-old man with mass-forming intrahepatic cholangiocarcinomas.
CT scan shows irregularly shaped mass with peripheral
enhancement, note three small peritumoral satellite nodules (arrow)

25. *MRI :
-The MR imaging features of mass-forming
cholangiocarcinoma are similar to its CT features
-The mass shows an irregular margin with high signal
intensity at T2 and with low signal intensity at T1
-Both the peripheral and the centripetal enhancement may
be more prominent at MR imaging than at CT
-In certain cases, prominent central enhancement can be
seen on the equilibrium phase or delayed phase MR
images, a finding that is similar to the enhancement
pattern seen at contrast-enhanced CT, the area of the
tumor with early enhancement and rapid washout
indicates active growth, whereas the central area is
composed mainly of loose connective tissue with an
abundant intercellular matrix

26. Typical MR imaging features of mass-forming cholangiocarcinoma, (a) Axial fat-
suppressed T2 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 show irregular, ragged rim enhancement (arrows
in b) with gradual centripetal enhancement (arrowheads in c)

27. b) Periductal infiltrating :
-Periductal infiltrating cholangiocarcinoma is
characterized by growth along a dilated or
narrowed bile duct without mass formation and
manifests as an elongated, spiculated, or
branchlike abnormality
*US :
-Appears as a small, masslike lesion or diffuse bile
duct thickening with or without obliteration of the
bile duct lumen depending on tumor extent

28. Periductal infiltrating intrahepatic cholangiocarcinoma is characterized
by tumor infiltration along the bile duct (arrow), it occasionally
involves the surrounding blood vessels or hepatic parenchyma

30. *CT & MRI :
-Diffuse periductal thickening and increased
enhancement due to tumor infiltration can be
seen, with an abnormally dilated or irregularly
narrowed duct and peripheral ductal dilatation
-This type of tumor is rare in intrahepatic
cholangiocarcinoma, but most hilar
cholangiocarcinomas are of this type
-In the periphery of the liver, a combination of the
periductal and mass-forming types is more
common than a purely periductal infiltrating
lesion

31. Periductal infiltrating intrahepatic cholangiocarcinoma in a 54-year-old man,
(a) CT scan shows segmental dilatation of the intrahepatic duct in segment
III (B3) only, multiple filling defects representing intrahepatic duct stones are
also noted, (b) CT scan obtained 1 cm inferior to a shows a low-attenuation
mass anterior to the left portal vein (arrow)

32. 55-year-old man with periductal-infiltrating intrahepatic and extrahepatic
cholangiocarcinoma, CT scan obtained at portal venous phase shows left
intrahepatic bile duct dilatation (curved arrow) and obliteration of bile ducts
in right hepatic lobe and hepatic hilum. Ill-defined, branchlike, low-
attenuating mass (straight arrows) represents periductal infiltrating
intrahepatic cholangiocarcinoma

33. Periductal infiltrating hilar cholangiocarcinoma, Coronal T2 shows
irregular ductal wall thickening along a narrowed hilar bile duct
(arrow)

34. Periductal infiltrating cholangiocarcinoma, (a) Axial T2 shows a dilated
peripheral intrahepatic duct with a slightly hyperintense lesion
around the duct (arrow), (b) Contrast-enhanced equilibrium phase
MR image shows periductal enhancement around the dilated
intrahepatic duct (arrowheads)

35. c) Intraductal growth :
-Imaging patterns include :
(a) diffuse and marked ductectasia with a grossly
visible papillary mass
(b) diffuse and marked ductectasia without a
visible mass
(c) an intraductal polypoid mass within localized
ductal dilatation
(d) intraductal castlike lesions within a mildly
dilated duct
(e) a focal stricture-like lesion with mild proximal
ductal dilatation

36. 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 recorded (eg, “periductal infiltrating + intraductal”)

38. -Intraductal cholangiocarcinoma may be classified
as either :
(1) Macroscopic
(2) Microscopic
-Microscopic lesions may represent the early form
of typical cholangiocarcinoma, whereas
macroscopic lesions may represent a distinct
pathologic entity, macroscopic lesions manifest
as either papillary or tubular polypoid lesions

39. (a) Diffuse and Marked Ductectasia with a
Grossly Visible Papillary Mass :
-The most distinguishable imaging pattern of the
first type of intraductal cholangiocarcinoma is
diffuse ductal dilatation with multifocal superficial
spreading papillary or plaquelike masses at CT
or MR imaging
*US :
-An intraductal polypoid lesion is echogenic
relative to the surrounding liver

40. *CT :
-At precontrast CT, an intraductal mass appears
as a lesion within the dilated bile duct that is
hypo- or isoattenuating relative to the
surrounding liver
-After contrast medium administration, the
intraductal tumor shows enhancement, this
lesion is usually confined to the bile duct wall, so
that the wall will appear intact at US and CT
-In some cases, only marked intrahepatic bile duct
dilatation with no obstructive mass or stricture
can be detected at imaging, these imaging
findings can be explained on the basis of
copious mucin production, because mucin is
usually anechoic at US and appears
isoattenuating relative to water at precontrast
CT, it is hard to detect at US or CT

41. Intraductal papillary neoplasm of the biliary tract with marked mucin
production, CT+C (a) and T2 (b) show a markedly dilated
intrahepatic duct with mural nodules or irregular wall thickening
(arrow)

42. (b) Diffuse and Marked Ductectasia without a
Visible Mass :
-In the second pattern of intraductal
cholangiocarcinoma, a diffuse and marked
ductectasia is present as in the first pattern, but
a grossly visible mass is not present at CT& MR
imaging
-This is either because of the micropapillary nature
of the tumor or because of the limited spatial
resolution of the imaging modalities

43. CT+C shows diffuse ductal dilatation in the left hepatic lobe through the
common bile duct, with no visible intraductal mass

44. (c) An Intraductal Polypoid Mass within
Localized Ductal Dilatation :
-The third pattern of intraductal tumors
manifests as localized ductal dilatation
with an intraductal mass
-An intraductal papillary mass is also usually
present, but mucin secretion is not
remarkable, so that the distal ductal
dilatation is not prominent

45. 53-year-old man with intraductal growing intrahepatic
cholangiocarcinoma, CT+C shows aneurysmally dilated left hepatic
bile ducts containing multiple fungating tumors and fluid in between,
peripheral bile ducts (arrow) are dilated

46. Intraductal papillary cholangiocarcinoma, MRCP shows an intraductal
polypoid mass with localized ductal dilatation (arrow)

47. (d) Intraductal Castlike Lesions within a
Mildly Dilated Duct :
-The fourth pattern is one of the most
difficult forms to diagnose correctly at
imaging
-It manifests as an area of mild ductal
dilatation filled with intraductal soft-tissue
material, which may show mild
enhancement at CT or MR imaging

48. Intraductal cholangiocarcinoma, (a) CT scan shows a soft-tissue
component filling a mildly dilated duct (arrow), (b) MRCP shows the
mildly dilated duct with irregularities that mimic impacted stones
(arrowheads)

49. (e) A Focal Stricture-like Lesion with Mild
Proximal Ductal Dilatation :
-The last pattern of intraductal
cholangiocarcinoma manifests as a focal
stricture-like lesion with mild proximal
ductal dilatation and no demonstrable
mass

50. Axial contrast-enhanced MR image show a focal stricture
(arrow) with mild ductal dilatation

51. 5-Staging : Bismuth-Corlette classification
-Classification system for perihilar cholangiocarcinoma
which is based on the extent of ductal infiltration
*Type I : Limited to the common hepatic duct below the
level of the confluence of the right and left hepatic ducts
*Type II : Involves the confluence of the right and left
hepatic ducts
*Type IIIa : Type II + extends to the bifurcation of
the right hepatic duct
*Type IIIb : Type II + extends to the bifurcation of
the left hepatic duct
*Type IV : Extending to the bifurcations of both right and
left hepatic ducts OR multifocal involvement

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

53. 6-Radiographic Features :
a) Dilated intrahepatic ducts
b) Hilar lesions
c) Peripheral Lesions
d) CT
e) MRCP

54. a) Dilated intrahepatic ducts with normal
extrahepatic ducts
b) Hilar lesions :
-Central obstruction
-Lesions are usually infiltrative so that a
mass is not usually apparent
-Encasement of portal veins causes
irregular enhancement by CT

55. Infiltrative hilar cholangiocarcinoma in a 59-year-old woman with
progressive jaundice, (a) Arterial-phase CT shows a well-enhancing,
thickened bile duct wall (arrows) at the hepatic hilar level,
(b) Cholangiogram shows complete obstruction at the hepatic hilar
level and severe strictures (arrows) that involve both hepatic ducts

56. Klatskin Tumor: arterial and portal venous phase

57. c) Peripheral Lesions :
-May present as a focal mass or be diffusely
infiltrative
-Retain contrast materials on delayed scans
(minor peripheral enhancement with
gradual enhancement centrally)
-Occasionally invade veins

58. 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)

59. d) CT :
The key findings to look for are >>
1-Delayed enhancement
2-Peripheral biliary dilatation
3-Capsular contraction
e) MRCP :
1-Short annular constricting lesion , 75%
2-Long stricture , 10 %
3-Intraluminal polypoid mass , 5 %

60. Delayed Enhancement

61. Capsular Retraction

62. 67-year-old man with Klatskin tumor, MRCP shows tumor involvement
of primary confluence of bile duct (arrow)