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(Radiographics. 2002;22:173-187.)
© RSNA, 2002


EDUCATION EXHIBIT

Cholangiocarcinoma: Pictorial Essay of CT and Cholangiographic Findings1

Joon Koo Han, MD, Byung Ihn Choi, MD, Ah Young Kim, MD, Su Kyung An, MD, Joon Woo Lee, MD, Tae Kyung Kim, MD and Sun-Whe Kim, MD

1 From the Departments of Radiology (J.K.H., B.I.C., A.Y.K., S.K.A., J.W.L., T.K.K.) and General Surgery (S.W.K.), Seoul National University College of Medicine, 28 Yongon-dong, Chongno-Gu, Seoul 110-744, Korea. Received March 15, 2001; revision requested May 3 and final revision received September 12; accepted September 17. Address correspondence to J.K.H. (e-mail: hanjk@radcom.snu.ac.kr).


    Abstract
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 
Cholangiocarcinomas that involve areas from the peripheral intrahepatic duct to the distal common duct have similar morphologic features, and traditional classification schemes based on the location of the involved ducts sometimes overlap. Nevertheless, cholangiocarcinoma is usually classified as either intrahepatic or extrahepatic, and intrahepatic cholangiocarcinoma is further classified as either peripheral or hilar. However, the distinction between peripheral intrahepatic cholangiocarcinoma and hilar cholangiocarcinoma is largely based on the site of origin. Therefore, in some tumors that arise peripheral to the secondary bifurcation of one of the hepatic ducts, clear differentiation between the two types of cholangiocarcinoma is not always possible. In addition, the distinction between hilar cholangiocarcinoma and extrahepatic cholangiocarcinoma is not clearly defined. The different biologic behaviors of the tumors seem to be caused by their varying locations and their size at the time of diagnosis. Further molecular or biochemical investigation is needed to support the "field theory," which states that all cholangiocarcinomas are biologically the same tumor originating from the same biliary epithelium.

© RSNA, 2002

Index Terms: Bile duct radiography, 76.122 • Bile ducts, CT, 76.1211 • Bile ducts, neoplasms, 76.321 • Bile ducts, stenosis or obstruction


    Introduction
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 
Cholangiocarcinoma is an adenocarcinoma that arises from the bile duct epithelium. It is the second most prevalent liver cancer after hepatocellular carcinoma. Cholangiocarcinoma is associated with clonorchiasis, intrahepatic stone disease, choledochal cyst, Caroli disease, and primary sclerosing cholangitis (1). It is usually classified as either intrahepatic or extrahepatic, and intrahepatic cholangiocarcinoma is further classified as either peripheral or hilar. In the surgery literature, a tumor that arises peripheral to the secondary bifurcation of the left or right hepatic duct is considered to be peripheral cholangiocarcinoma, whereas a tumor that arises from one of the hepatic ducts or the bifurcation of the common hepatic duct is considered to be hilar cholangiocarcinoma (Klatskin tumor). These three types of cholangiocarcinoma—extrahepatic, peripheral intrahepatic, and hilar intrahepatic—are traditionally regarded as distinct disease entities clinically, therapeutically, and radiologically.

However, this classification scheme is controversial. In previous large series of patients with bile duct cancer, hilar cholangiocarcinoma was classified as extrahepatic bile duct cancer by some authors (2). Furthermore, some bile duct tumors involve both intrahepatic and extrahepatic ducts, which makes clear-cut classification difficult.

Peripheral cholangiocarcinoma classically manifests as a large, well-defined hepatic mass with lobulated margins and peripheral rim enhancement at both contrast material–enhanced spiral computed tomography (CT) and dynamic magnetic resonance (MR) imaging. Although CT provides better anatomic detail (eg, depicting vascular or extrahepatic invasion) and more accurate staging (3), MR imaging demonstrates more conspicuous contrast enhancement, which facilitates the detection and evaluation of hepatic parenchymal changes peripheral to the tumor. However, the two modalities are equally effective in the detection and correct diagnosis of the tumor.

Hilar cholangiocarcinoma typically takes one of three shapes: infiltrative, exophytic, or polypoid. Most extrahepatic cholangiocarcinomas are infiltrative, causing a focal stricture of the bile duct, whereas papillary carcinoma in the form of an intraductal polypoid mass is occasionally reported. Thus, infiltrative and polypoid hilar cholangiocarcinoma have a shape similar to that of extrahepatic cholangiocarcinoma.

Traditionally, tumors accompanying biliary dilatation have been evaluated with direct cholangiography (percutaneous transhepatic or endoscopic retrograde cholangiography). However, recent technologic developments have led to rapid improvement in the quality of MR cholangiography. This imaging modality is diagnostic in more than 90% of patients with malignant hilar strictures. Furthermore, MR cholangiography together with axial T1- and T2-weighted imaging may allow identification, characterization, and staging of these lesions (4,5).

There is little resemblance between peripheral cholangiocarcinoma and cholangiocarcinoma arising from the large ducts. However, recent studies have described intraductal papillary cholangiocarcinomas arising from peripheral intrahepatic ducts or from large intrahepatic ducts near the secondary confluence and having gross pathologic features identical to those of papillary cholangiocarcinoma of the extrahepatic bile duct or hepatic hilum. Furthermore, some authors have encountered cases of an initial manifestation of peripheral cholangiocarcinoma that subsequently recurred as intraductal papillary cholangiocarcinoma (and vice versa). This finding strongly suggests that all cholangiocarcinomas are similar, much like urothelial tumors of the kidney, renal pelvis, ureter, and bladder (6,7).

The classification scheme for primary liver cancer proposed by the Liver Cancer Study Group of Japan (8) divides intrahepatic cholangiocarcinomas into three types based on macroscopic appearance: mass-forming, periductal infiltrating, and intraductal (Fig 1). The mass-forming type is a classic intrahepatic cholangiocarcinoma that manifests as a large, lobulated mass with low attenuation at CT. Periductal infiltrating intrahepatic cholangiocarcinoma is the most common type of cholangiocarcinoma in the hilar area. It is almost identical to infiltrating extrahepatic cholangiocarcinoma both macroscopically and radiologically. Intraductal intrahepatic cholangiocarcinoma is morphologically similar to papillary cholangiocarcinoma of the large bile duct in the hepatic hilum and extrahepatic area.



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Figure 1a.   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 recorded (eg, "periductal infiltrating + intraductal"). (Fig 1a-Fig 1c reprinted, with permission, from reference 8.)

 


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Figure 1b.   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 recorded (eg, "periductal infiltrating + intraductal"). (Fig 1a-Fig 1c reprinted, with permission, from reference 8.)

 


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Figure 1c.   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 recorded (eg, "periductal infiltrating + intraductal"). (Fig 1a-Fig 1c reprinted, with permission, from reference 8.)

 
In this article, we discuss and illustrate radiologic findings with pathologic correlation in a wide spectrum of cholangiocarcinomas with emphasis on their common radiologic-pathologic features regardless of the site of origin.


    Intrahepatic Cholangiocarcinoma
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 
Mass-forming Intrahepatic Cholangiocarcinoma
Mass-forming intrahepatic cholangiocarcinoma (peripheral cholangiocarcinoma) is the most common type of intrahepatic cholangiocarcinoma. At gross pathologic examination, peripheral cholangiocarcinoma typically manifests as a large, white tumor with dense fibrosis. Peripheral cholangiocarcinomas are usually large because they are rarely symptomatic early in their course. At CT, peripheral cholangiocarcinoma has been described as an irregular mass with markedly low attenuation, minimal peripheral enhancement, and focal dilatation of intrahepatic ducts around the tumor. At spiral CT, peripheral cholangiocarcinoma usually demonstrates thin, incomplete rim enhancement during both the arterial and portal venous phases. The central part of the tumor does not enhance during these phases, whereas there may be prolonged enhancement at delayed-phase CT. Frequently noted ancillary findings in peripheral cholangiocarcinoma include capsular retraction and dilatation and thickening of the peripheral intrahepatic ducts (especially when associated with clonorchiasis) (Fig 2) (9,10).



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Figure 2a.   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). (c) Photograph of the pathologic specimen obtained at segmentectomy reveals a lobulated, yellow-white mass without encapsulation. Note also the markedly thickened bile duct walls caused by periductal fibrosis (arrows), which is a common finding in clonorchiasis. (d) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows adenocarcinoma with extensive necrosis (*). As in c, the mass is not encapsulated. Invasion into the hepatic parenchyma is also noted (arrow).

 


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Figure 2b.   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). (c) Photograph of the pathologic specimen obtained at segmentectomy reveals a lobulated, yellow-white mass without encapsulation. Note also the markedly thickened bile duct walls caused by periductal fibrosis (arrows), which is a common finding in clonorchiasis. (d) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows adenocarcinoma with extensive necrosis (*). As in c, the mass is not encapsulated. Invasion into the hepatic parenchyma is also noted (arrow).

 


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Figure 2c.   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). (c) Photograph of the pathologic specimen obtained at segmentectomy reveals a lobulated, yellow-white mass without encapsulation. Note also the markedly thickened bile duct walls caused by periductal fibrosis (arrows), which is a common finding in clonorchiasis. (d) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows adenocarcinoma with extensive necrosis (*). As in c, the mass is not encapsulated. Invasion into the hepatic parenchyma is also noted (arrow).

 


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Figure 2d.   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). (c) Photograph of the pathologic specimen obtained at segmentectomy reveals a lobulated, yellow-white mass without encapsulation. Note also the markedly thickened bile duct walls caused by periductal fibrosis (arrows), which is a common finding in clonorchiasis. (d) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin [H-E] stain) shows adenocarcinoma with extensive necrosis (*). As in c, the mass is not encapsulated. Invasion into the hepatic parenchyma is also noted (arrow).

 
Intraductal Intrahepatic Cholangiocarcinoma
Intraductal intrahepatic cholangiocarcinoma has a better prognosis than other types of cholangiocarcinoma and, in some instances, a peculiar growth pattern characterized by superficial mucosal spreading. At histologic analysis, this type of tumor is generally considered to be papillary cholangiocarcinoma. At CT, characteristic features include segmental or lobar dilatation of the intrahepatic bile duct with higher attenuation than that of bile (Fig 3). An obstructing mass is occasionally seen as a low-attenuation lesion when it is larger than 1 cm (11). Because of its peculiar growth patterns, this type of cholangiocarcinoma is thought to be a distinct entity rather than an early manifestation of the more typical mass-forming intrahepatic cholangiocarcinoma; in some patients, however, papillary cholangiocarcinoma demonstrates an intraductal component as well as stromal invasion (Fig 4). Papillary cholangiocarcinoma constitutes 8%–18% of all resected cholangiocarcinomas (11).



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Figure 3a.   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 Fig 3c reprinted, with permission, from reference 11.)

 


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Figure 3b.   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 Fig 3c reprinted, with permission, from reference 11.)

 


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Figure 3c.   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 Fig 3c reprinted, with permission, from reference 11.)

 


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Figure 4a.   Intraductal intrahepatic cholangiocarcinoma and mass-forming cholangiocarcinoma with an intraductal component in a 78-year-old man. (a) CT scan shows two low-attenuation masses in segments III and VII of the liver (open arrows). Note the mild ductal dilatation adjacent to the tumors (solid arrows). (b) Photograph of the resected specimen obtained at left lateral segmentectomy reveals a 3 x 1.5-cm papillary tumor confined to the bile duct. There is no invasion into the hepatic parenchyma. (c) Photograph of the resected specimen obtained at right lobectomy reveals a 7.4 x 4-cm yellow-white mass. Note also the dilated intrahepatic duct filled with multiple papillary tumors (arrow). (d) Low-power photomicrograph (original magnification, x40; H-E stain) shows a dilated duct filled with a papillary tumor. In most areas, the tumors were confined within the ducts. Some focal areas of stromal invasion were also noted.

 


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Figure 4b.   Intraductal intrahepatic cholangiocarcinoma and mass-forming cholangiocarcinoma with an intraductal component in a 78-year-old man. (a) CT scan shows two low-attenuation masses in segments III and VII of the liver (open arrows). Note the mild ductal dilatation adjacent to the tumors (solid arrows). (b) Photograph of the resected specimen obtained at left lateral segmentectomy reveals a 3 x 1.5-cm papillary tumor confined to the bile duct. There is no invasion into the hepatic parenchyma. (c) Photograph of the resected specimen obtained at right lobectomy reveals a 7.4 x 4-cm yellow-white mass. Note also the dilated intrahepatic duct filled with multiple papillary tumors (arrow). (d) Low-power photomicrograph (original magnification, x40; H-E stain) shows a dilated duct filled with a papillary tumor. In most areas, the tumors were confined within the ducts. Some focal areas of stromal invasion were also noted.

 


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Figure 4c.   Intraductal intrahepatic cholangiocarcinoma and mass-forming cholangiocarcinoma with an intraductal component in a 78-year-old man. (a) CT scan shows two low-attenuation masses in segments III and VII of the liver (open arrows). Note the mild ductal dilatation adjacent to the tumors (solid arrows). (b) Photograph of the resected specimen obtained at left lateral segmentectomy reveals a 3 x 1.5-cm papillary tumor confined to the bile duct. There is no invasion into the hepatic parenchyma. (c) Photograph of the resected specimen obtained at right lobectomy reveals a 7.4 x 4-cm yellow-white mass. Note also the dilated intrahepatic duct filled with multiple papillary tumors (arrow). (d) Low-power photomicrograph (original magnification, x40; H-E stain) shows a dilated duct filled with a papillary tumor. In most areas, the tumors were confined within the ducts. Some focal areas of stromal invasion were also noted.

 


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Figure 4d.   Intraductal intrahepatic cholangiocarcinoma and mass-forming cholangiocarcinoma with an intraductal component in a 78-year-old man. (a) CT scan shows two low-attenuation masses in segments III and VII of the liver (open arrows). Note the mild ductal dilatation adjacent to the tumors (solid arrows). (b) Photograph of the resected specimen obtained at left lateral segmentectomy reveals a 3 x 1.5-cm papillary tumor confined to the bile duct. There is no invasion into the hepatic parenchyma. (c) Photograph of the resected specimen obtained at right lobectomy reveals a 7.4 x 4-cm yellow-white mass. Note also the dilated intrahepatic duct filled with multiple papillary tumors (arrow). (d) Low-power photomicrograph (original magnification, x40; H-E stain) shows a dilated duct filled with a papillary tumor. In most areas, the tumors were confined within the ducts. Some focal areas of stromal invasion were also noted.

 
Periductal Infiltrating Intrahepatic Cholangiocarcinoma
Periductal infiltrating intrahepatic cholangiocarcinoma is radiologically and pathologically identical to infiltrating hilar cholangiocarcinoma (Klatskin tumor) but has a different location (ie, peripheral to the secondary confluence). Very small fibrotic tumors cause segmental dilatation of the bile ducts. Unlike with intraductal intrahepatic chol-angiocarcinoma, the bile ducts are isoattenuating relative to water, unless secondary biliary stones or sludge is formed (Fig 5). In the later stage, the tumor may invade the hepatic parenchyma and hepatic hilum. In such cases, the tumor is transformed into exophytic hilar cholangiocarcinoma.



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Figure 5a.   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). (c) Percutaneous transhepatic cholangiogram shows a short narrowed segment at the B3 orifice. Multiple intrahepatic duct stones are also noted. Fluoroscopically guided forceps biopsy revealed cholangiocarcinoma. (d) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen obtained at left lobectomy reveals well-differentiated adenocarcinoma confined to periductal connective tissue.

 


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Figure 5b.   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). (c) Percutaneous transhepatic cholangiogram shows a short narrowed segment at the B3 orifice. Multiple intrahepatic duct stones are also noted. Fluoroscopically guided forceps biopsy revealed cholangiocarcinoma. (d) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen obtained at left lobectomy reveals well-differentiated adenocarcinoma confined to periductal connective tissue.

 


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Figure 5c.   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). (c) Percutaneous transhepatic cholangiogram shows a short narrowed segment at the B3 orifice. Multiple intrahepatic duct stones are also noted. Fluoroscopically guided forceps biopsy revealed cholangiocarcinoma. (d) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen obtained at left lobectomy reveals well-differentiated adenocarcinoma confined to periductal connective tissue.

 


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Figure 5d.   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). (c) Percutaneous transhepatic cholangiogram shows a short narrowed segment at the B3 orifice. Multiple intrahepatic duct stones are also noted. Fluoroscopically guided forceps biopsy revealed cholangiocarcinoma. (d) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen obtained at left lobectomy reveals well-differentiated adenocarcinoma confined to periductal connective tissue.

 
Hilar Cholangiocarcinoma
Although hilar cholangiocarcinomas have been subsumed under the heading of intrahepatic cholangiocarcinoma, their clinical and radiologic features as well as surgical management are more similar to those of extrahepatic cholangiocarcinomas. Therefore, in some textbooks, both entities are described as "large duct cancer" or "carcinoma of the bile duct" (1).

Hilar cholangiocarcinomas account for more than 50% of all large bile duct malignancies. The middle and distal portions of the common bile duct are affected in about 17% and 18% of these cases, respectively; in about 7% of cases, there is diffuse cancerous involvement (2,6,7). Tumors originating from a large bile duct are in a critical location and are discovered early due to the presence of jaundice or cholangitis. Thus, these tumors are usually very small. Conversely, tumors originating from small bile ducts do not cause significant biliary obstruction until the late stage, when the tumor itself or metastatic hilar lymphadenopathy causes obstruction of the common hepatic duct. Therefore, most peripheral cholangiocarcinomas are large at the time of diagnosis.

Infiltrating Hilar Cholangiocarcinoma. Infiltrating hilar cholangiocarcinoma is the most common type of hilar cholangiocarcinoma (over 70% of cases). At contrast-enhanced CT, infiltrating tumors are seen as a focally thickened ductal wall obliterating the lumen (Fig 6). About 80% of these tumors are hyperattenuating relative to the liver. At pathologic analysis, infiltrating hilar cholangiocarcinoma manifests as a sclerotic lesion with abundant fibrous tissue (12).



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Figure 6a.   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.

 


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Figure 6b.   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.

 


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Figure 6c.   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.

 


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Figure 6d.   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.

 
Exophytic Hilar Cholangiocarcinoma. Exophytic hilar cholangiocarcinoma manifests as a large, low-attenuation mass with peripheral rim enhancement, findings that are similar to those in peripheral cholangiocarcinoma. It can be difficult or even impossible to ascertain whether the carcinoma arises at the main hepatic juncture or represents a peripheral cholangiocarcinoma that secondarily obliterates the hilar area (Figs 7, 8). The lesion may also represent infiltrating intrahepatic cholangiocarcinoma (Fig 5) that involves the hepatic hilum by intraductal spreading and the hepatic parenchyma by direct invasion.



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Figure 7a.   Exophytic hilar cholangiocarcinoma. Arterial (a) and portal-phase (b) CT scans show a 4-cm low-attenuation mass with peripheral enhancement at the hepatic hilum, mainly in segment IV (arrow). Both intrahepatic ducts are dilated. Two pigtail catheters were inserted for drainage. (c) Cholangiogram reveals obliteration of the left hepatic duct (arrow), but the right secondary confluence is preserved. In such cases, it is difficult to ascertain whether the tumor originates from the left hepatic duct and demonstrates exophytic growth or originates from a small branch of segment 4 and exhibits subsequent involvement of the hepatic hilum.

 


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Figure 7b.   Exophytic hilar cholangiocarcinoma. Arterial (a) and portal-phase (b) CT scans show a 4-cm low-attenuation mass with peripheral enhancement at the hepatic hilum, mainly in segment IV (arrow). Both intrahepatic ducts are dilated. Two pigtail catheters were inserted for drainage. (c) Cholangiogram reveals obliteration of the left hepatic duct (arrow), but the right secondary confluence is preserved. In such cases, it is difficult to ascertain whether the tumor originates from the left hepatic duct and demonstrates exophytic growth or originates from a small branch of segment 4 and exhibits subsequent involvement of the hepatic hilum.

 


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Figure 7c.   Exophytic hilar cholangiocarcinoma. Arterial (a) and portal-phase (b) CT scans show a 4-cm low-attenuation mass with peripheral enhancement at the hepatic hilum, mainly in segment IV (arrow). Both intrahepatic ducts are dilated. Two pigtail catheters were inserted for drainage. (c) Cholangiogram reveals obliteration of the left hepatic duct (arrow), but the right secondary confluence is preserved. In such cases, it is difficult to ascertain whether the tumor originates from the left hepatic duct and demonstrates exophytic growth or originates from a small branch of segment 4 and exhibits subsequent involvement of the hepatic hilum.

 


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Figure 8a.   Infiltrating hilar cholangiocarcinoma with early-stage exophytic growth. (a) Percutaneous transhepatic cholangiogram shows hilar cholangiocarcinoma originating mainly from the right hepatic duct (arrows). (b) CT scan shows the tumor as a thin line of high attenuation (arrows) anterior to the right portal vein. (c) Photograph of the resected specimen cut along the right hepatic duct shows a whitish tumor invading the hepatic parenchyma. This finding, which was not seen at CT, represents early-stage exophytic growth. (d) High-power photomicrograph (original magnification, x100; H-E stain) of the resected specimen reveals moderately differentiated adenocarcinoma. (Fig 8a-Fig 8c reprinted, with permission, from reference 12.)

 


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Figure 8b.   Infiltrating hilar cholangiocarcinoma with early-stage exophytic growth. (a) Percutaneous transhepatic cholangiogram shows hilar cholangiocarcinoma originating mainly from the right hepatic duct (arrows). (b) CT scan shows the tumor as a thin line of high attenuation (arrows) anterior to the right portal vein. (c) Photograph of the resected specimen cut along the right hepatic duct shows a whitish tumor invading the hepatic parenchyma. This finding, which was not seen at CT, represents early-stage exophytic growth. (d) High-power photomicrograph (original magnification, x100; H-E stain) of the resected specimen reveals moderately differentiated adenocarcinoma. (Fig 8a-Fig 8c reprinted, with permission, from reference 12.)

 


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Figure 8c.   Infiltrating hilar cholangiocarcinoma with early-stage exophytic growth. (a) Percutaneous transhepatic cholangiogram shows hilar cholangiocarcinoma originating mainly from the right hepatic duct (arrows). (b) CT scan shows the tumor as a thin line of high attenuation (arrows) anterior to the right portal vein. (c) Photograph of the resected specimen cut along the right hepatic duct shows a whitish tumor invading the hepatic parenchyma. This finding, which was not seen at CT, represents early-stage exophytic growth. (d) High-power photomicrograph (original magnification, x100; H-E stain) of the resected specimen reveals moderately differentiated adenocarcinoma. (Fig 8a-Fig 8c reprinted, with permission, from reference 12.)

 


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Figure 8d.   Infiltrating hilar cholangiocarcinoma with early-stage exophytic growth. (a) Percutaneous transhepatic cholangiogram shows hilar cholangiocarcinoma originating mainly from the right hepatic duct (arrows). (b) CT scan shows the tumor as a thin line of high attenuation (arrows) anterior to the right portal vein. (c) Photograph of the resected specimen cut along the right hepatic duct shows a whitish tumor invading the hepatic parenchyma. This finding, which was not seen at CT, represents early-stage exophytic growth. (d) High-power photomicrograph (original magnification, x100; H-E stain) of the resected specimen reveals moderately differentiated adenocarcinoma. (Fig 8a-Fig 8c reprinted, with permission, from reference 12.)

 
Polypoid Hilar Cholangiocarcinoma. Polypoid hilar cholangiocarcinoma manifests as an intraductal soft-tissue mass that is hypoattenuating relative to the hepatic parenchyma (6,7,12). It has the same gross and histologic features as intraductal intrahepatic cholangiocarcinoma (Figs 9, 10). The tumors are frequently multiple or disseminated within the biliary system and involve both the intrahepatic and extrahepatic bile ducts. Their site of origin determines the presenting symptoms (jaundice in large duct lesions, incidentally found segmental ductal dilatation in intrahepatic lesions).



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Figure 9a.   Polypoid hilar cholangiocarcinoma. (a, b) Delayed-phase CT scans (b obtained at a lower level than a) show a soft-tissue mass (arrow) within a dilated left hepatic duct and common bile duct. (c) Cholangiogram shows a polypoid mass at the confluence level (arrows). (d) Photograph of the resected specimen obtained at left lobectomy and caudate lobectomy reveals an extensive polypoid mass (arrow) within the common bile duct (CBD). LHD = left hepatic duct, RHD = right hepatic duct.

 


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Figure 9b.   Polypoid hilar cholangiocarcinoma. (a, b) Delayed-phase CT scans (b obtained at a lower level than a) show a soft-tissue mass (arrow) within a dilated left hepatic duct and common bile duct. (c) Cholangiogram shows a polypoid mass at the confluence level (arrows). (d) Photograph of the resected specimen obtained at left lobectomy and caudate lobectomy reveals an extensive polypoid mass (arrow) within the common bile duct (CBD). LHD = left hepatic duct, RHD = right hepatic duct.

 


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Figure 9c.   Polypoid hilar cholangiocarcinoma. (a, b) Delayed-phase CT scans (b obtained at a lower level than a) show a soft-tissue mass (arrow) within a dilated left hepatic duct and common bile duct. (c) Cholangiogram shows a polypoid mass at the confluence level (arrows). (d) Photograph of the resected specimen obtained at left lobectomy and caudate lobectomy reveals an extensive polypoid mass (arrow) within the common bile duct (CBD). LHD = left hepatic duct, RHD = right hepatic duct.

 


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Figure 9d.   Polypoid hilar cholangiocarcinoma. (a, b) Delayed-phase CT scans (b obtained at a lower level than a) show a soft-tissue mass (arrow) within a dilated left hepatic duct and common bile duct. (c) Cholangiogram shows a polypoid mass at the confluence level (arrows). (d) Photograph of the resected specimen obtained at left lobectomy and caudate lobectomy reveals an extensive polypoid mass (arrow) within the common bile duct (CBD). LHD = left hepatic duct, RHD = right hepatic duct.

 


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Figure 10a.   Polypoid hilar cholangiocarcinoma in a 64-year-old man. (a) Contrast-enhanced CT scan obtained with iodized oil (Lipiodol; Guerbet, Roissy, France) 10 years earlier for mass-forming intrahepatic cholangiocarcinoma reveals a 4-cm mass in segment VI of the liver. The mass is partially enhanced. Segment VI segmentectomy was performed, and pathologic analysis helped confirm the presence of typical peripheral cholangiocarcinoma (mass-forming intrahepatic cholangiocarcinoma). Follow-up CT demonstrated slowly progressive intrahepatic duct dilatation and atrophy of the right hepatic lobe. (b) Portal-phase CT scan shows diffuse dilatation of the intrahepatic duct, especially in segments IV and VIII. In addition, a soft-tissue mass is noted in the common bile duct (arrow). (c) Percutaneous transhepatic cholangiogram reveals a papillary mass at the level of the hepatic hilum (arrows), presumably arising from the right hepatic duct.

 


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Figure 10b.   Polypoid hilar cholangiocarcinoma in a 64-year-old man. (a) Contrast-enhanced CT scan obtained with iodized oil (Lipiodol; Guerbet, Roissy, France) 10 years earlier for mass-forming intrahepatic cholangiocarcinoma reveals a 4-cm mass in segment VI of the liver. The mass is partially enhanced. Segment VI segmentectomy was performed, and pathologic analysis helped confirm the presence of typical peripheral cholangiocarcinoma (mass-forming intrahepatic cholangiocarcinoma). Follow-up CT demonstrated slowly progressive intrahepatic duct dilatation and atrophy of the right hepatic lobe. (b) Portal-phase CT scan shows diffuse dilatation of the intrahepatic duct, especially in segments IV and VIII. In addition, a soft-tissue mass is noted in the common bile duct (arrow). (c) Percutaneous transhepatic cholangiogram reveals a papillary mass at the level of the hepatic hilum (arrows), presumably arising from the right hepatic duct.

 


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Figure 10c.   Polypoid hilar cholangiocarcinoma in a 64-year-old man. (a) Contrast-enhanced CT scan obtained with iodized oil (Lipiodol; Guerbet, Roissy, France) 10 years earlier for mass-forming intrahepatic cholangiocarcinoma reveals a 4-cm mass in segment VI of the liver. The mass is partially enhanced. Segment VI segmentectomy was performed, and pathologic analysis helped confirm the presence of typical peripheral cholangiocarcinoma (mass-forming intrahepatic cholangiocarcinoma). Follow-up CT demonstrated slowly progressive intrahepatic duct dilatation and atrophy of the right hepatic lobe. (b) Portal-phase CT scan shows diffuse dilatation of the intrahepatic duct, especially in segments IV and VIII. In addition, a soft-tissue mass is noted in the common bile duct (arrow). (c) Percutaneous transhepatic cholangiogram reveals a papillary mass at the level of the hepatic hilum (arrows), presumably arising from the right hepatic duct.

 

    Extrahepatic Cholangiocarcinoma
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 
Infiltrating Extrahepatic Cholangiocarcinoma
Infiltrating extrahepatic cholangiocarcinoma is the most common type of cholangiocarcinoma involving the extrahepatic bile duct. It manifests as a high-attenuation mass or thickened wall at the site of biliary obstruction and has radiologic and pathologic features that are identical to those of hilar cholangiocarcinoma. On serial CT scans, the dilated extrahepatic bile duct suddenly disappears and the lumen is replaced by a small, high-attenuation mass encircling the lumen (Fig 11).



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Figure 11a.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 


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Figure 11b.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 


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Figure 11c.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 


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Figure 11d.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 


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Figure 11e.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 


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Figure 11f.   Infiltrating extrahepatic cholangiocarcinoma involving the midportion of the common bile duct. (a) CT scan demonstrates a dilated common bile duct with minimal wall thickening (arrow). The thickened wall is hyperattenuating relative to the wall of the gallbladder. (b) Subsequent CT scan shows more pronounced luminal narrowing and wall thickening (arrow) than was seen in a. (c) CT scan demonstrates thickening of the ductal wall and almost total obliteration of the lumen in the intrapancreatic portion of the common bile duct (arrow). A small, high-attenuation mass is seen encircling the duct. (d) Cholangiogram obtained during percutaneous transhepatic biliary drainage shows complete obstruction of the proximal common bile duct. (e) Low-power photomicrograph (original magnification, x20; H-E stain) of the resected specimen shows focal thickening of the bile duct wall adjacent to the tumor (solid arrows). Note also the pancreatic invasion by the tumor (open arrows), which consists mainly of fibrosis with scattered intratumoral cells. (f) Higher-power photomicrograph (original magnification, x100; H-E stain) shows dense fibrosis and scattered islands of tumor cells.

 
Polypoid Extrahepatic Cholangiocarcinoma
Polypoid extrahepatic cholangiocarcinoma manifests as a low-attenuation mass within the dilated bile duct and is identical to masses in the hilar area or intrahepatic ducts. This tumor frequently demonstrates extensive superficial spreading, resulting in diffuse involvement, which has been noted in 7% of cases of polypoid extrahepatic cholangiocarcinoma in large series (Fig 12) (2). The true extent of the tumor is difficult to determine, even with high-quality cholangiography. Therefore, choledochoscopy and biopsy are often necessary before curative resection is performed. The benign counterpart of polypoid extrahepatic cholangiocarcinoma is known as papilloma or papillomatosis (1).



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Figure 12a.   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.

 


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Figure 12b.   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.

 


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Figure 12c.   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.

 


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Figure 12d.   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.

 


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Figure 12e.   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.

 

    Conclusions
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 
Cholangiocarcinoma involving areas from the peripheral intrahepatic duct to the distal common duct have similar morphologic features. Traditional classification schemes based on the location of the involved ducts sometimes overlap. The different biologic behavior of the tumors seems to be caused by their varying locations and their size at the time of diagnosis. Further molecular or biochemical investigation is needed to support the "field theory," which states that all cholangiocarcinomas are biologically the same tumor originating from the same biliary epithelium.


    Footnotes
 
Abbreviation: H-E = hematoxylin-eosin


    References
 Top
 Abstract
 Introduction
 Intrahepatic Cholangiocarcinoma
 Extrahepatic Cholangiocarcinoma
 Conclusions
 References
 

  1. Sherlock S, Dooley J. Disease of the liver and biliary system 10th ed. London, England: Blackwell, 1997; 642-649.
  2. Tomkins RK, Saunders KD, Roslyn JJ, et al. Changing patterns in diagnosis and management of bile duct cancer. Ann Surg 1990; 211:614-621.[Medline]
  3. Zhang Y, Uchida M, Abe T, Nishimura H, Hayabuchi N, Nakashima Y. Intrahepatic peripheral cholangiocarcinoma: comparison of dynamic CT and dynamic MRI. J Comput Assist Tomogr 1999; 23:670-677.[CrossRef][Medline]
  4. Pavon P, Laghi A, Passariello R. MR cholangiopancreatography in malignant biliary obstruction. Semin Ultrasound CT MR 1999; 20:317-323.[CrossRef][Medline]
  5. Zidi SH, Prat F, Le Guen O, Rondeau Y, Pelletier G. Performance characteristics of magnetic resonance cholangiography in the staging of malignant hilar strictures. Gut 2000; 46:103-106.[Abstract/Free Full Text]
  6. Choi BI, Han JK, Kim TK. Benign and malignant tumors of the biliary tree. In: Gazelle SG, eds. Hepatobiliary and pancreatic radiology. New York, NY: Thieme, 1998; 630-676.
  7. Choi BI, Han JK, Kim TK. Diagnosis and staging of cholangiocarcinoma by computed tomography. In: Meyers MA, eds. Neoplasms of the digestive tract: imaging, staging and management. Philadelphia, Pa: Lippincott-Raven, 1998; 503-516.
  8. Liver Cancer Study Group of Japan. Classification of primary liver cancer Tokyo, Japan: Kanehara, 1997; 6-8.
  9. Kim TK, Choi BI, Han JK, Jang HJ, Cho SG, Han MC. Peripheral cholangiocarcinoma of the liver: two-phase spiral CT findings. Radiology 1997; 204:539-543.[Abstract/Free Full Text]
  10. Lim JH, Ko YT, Lee DH, Kim SY. Clonorchiasis: sonographic findings in 59 proved cases. AJR Am J Roentgenol 1989; 152:761-764.[Abstract/Free Full Text]
  11. Lee JW, Han JK, Kim TK, et al. CT features of intraductal intrahepatic cholangiocarcinoma. AJR Am J Roentgenol 2000; 175:721-725.[Abstract/Free Full Text]
  12. Han JK, Choi BI, Kim TK, Kim SW, Han MC, Yeon KM. Hilar cholangiocarcinoma: thin-section spiral CT findings with cholangiographic correlation. RadioGraphics 1997; 17:1475-1485.[Abstract]



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