Cholangiocarcinoma: appearances and mimics

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1 Cholangiocarcinoma: appearances and mimics Poster No.: C-1572 Congress: ECR 2011 Type: Educational Exhibit Authors: C. Cardenas Valencia, J. Fernandez Jara, J. Cubero Carralero, B. Corral Ramos, P. Perez Martin, J. Romero, M. Morales Garcia, A. Villalba Garcia, P. Borrego Jimenez; Leganes /Madrid/ES Keywords: Neoplasia, Computer Applications-Detection, diagnosis, MR, CT, Biliary Tract / Gallbladder DOI: /ecr2011/C-1572 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 38

2 Learning objectives Review the features of cholangiocarcinoma in terms of epidemiologic and pathologic considerations, risk factors, classification types and prognosis. Exhibit the spectrum of presentation of cholangiocarcinoma in CT and MR. Show CT and MR imaging of several neoplastic and nonneoplastic pathologies, that can have a similar imaging presentation as cholangiocarcinoma. Background INTRODUCTION Cholangiocarcinoma is a malignant tumor arising from the biliary tract. Risk factors for cholangiocarcinoma include: -Liver flukes. -Hepatolithiasis. -Viral infection (HIV, HBV, VCV). -Congenital biliopancreatic anomalies. -Cirrhosis. -Environmental or occupational toxin (thorotrast...). -Biliary tract-enteric drainage procedures. Most cholangiocarcinomas are adenocarcinomas with abundant fibrous stroma. Page 2 of 38

3 Cholangiocarinoma present a wide spectrum of imaging findings. There are several inflammatory and neoplasic lesions that mimics cholangiocarcinoma. Early diagnosis of cholangiocarcinoma is difficult and the prognosis is poor. TRADITIONALLY CLASSIFICATION OF CHOLANGIOCARCINOMA* (Figure 1) Peripheral intrahepatic: -Distal to the secondary bifurcation of the left or right hepatic duct. Hilar intrahepatic (Klastkin): -Involves hepatic ducts or the common hepatic duct. Extrahepatic: -Involves common bile duct. *Controversial classification. MORPHOLOGIC CLASSIFICATION CHOLANGIOCARCINOMA* (Figure 2) Based on radiologic-pathologic correlation. Useful for assess tumor dissemination, prognosis and surgical approach. Page 3 of 38

4 -Mass-forming type. -Periductal infiltrating type. -Intraductal growing type. *Classification system proposed by the Liver Cancer Study Group of Japan. Images for this section: Page 4 of 38

5 Fig. 1: Traditionally classification cholangiocarcinoma. Page 5 of 38

6 Fig. 2: Morphologic classification system proposed by the Liver Cancer Study Group of Japan. Page 6 of 38

7 Imaging findings OR Procedure details MASS-FORMING CHOLANGIOCARCINOMA (Figures 1, 2, 3 and 4) Homogenous mass with an irregular and well-defined margin. CT: -Homogenous attenuation. -Irregular peripheral enhancement. -Gradual centripetal enhancement*. -Capsular retraction. -Satellite nodules. MR: -Low signal intensity at T1. -High signal intensity at T2. -Irregular peripheral enhancement. -Gradual centripetal enhancement*. *Centripetal enhancement can be seen on the equilibrium phase or in the delayed phase. PERIDUCTAL INFILTRATING TYPE Page 7 of 38

8 (Figures 5 and 6) Growth along a dilated or narrowed bile duct without mass formation. CT and MR: -Diffuse periductal thickening and increased enhancement. -Dilated or irregularly narrowed duct. -Peripheral ductal dilatation. Most hiliar cholangiocarcinomas are of this type. Combination of the periductal and mass-forming types is common in the periphery of the liver. INTRADUCTAL TYPE (Figures 7, 8, 9 and 10) It has a variety of imaging patterns, grows slowly and has the best prognosis. Patterns: -Diffuse and marked ductectasia with a visible papillary mass. -Diffuse and marked ductectasia without a visible mass. -An intraductal polypoid mass within localized ductal dilatation. -Intraductal castlike lesions within a mildly dilated duct. -Focal stricture-like lesion with mild proximal ductal dilatation. CT and RM: Page 8 of 38

9 -Diffuse or localized ductal dilatation*. -Enhancement intraductal mass or lesions*. *According to the pattern. MIMICS OF CHOLANGIOCARCINOMA I. Primary sclerosing cholangitis II. Recurrent pyogenic Cholangitis III. Mirizzi syndrome IV. Hepathocarcinoma V. Biliary stones I. PRIMARY SCLEROSING CHOLANGITIS (Figures 11 and 12) Idiopathic, chronic, diffuse fibrosing inflammatory disease of the extra and intrahepatic bile ducts. Leads to bile ducts obliteration, cholestasis and biliary cirrhosis. Possible autoimmune origin. Association with inflammatory bowel disease (60-70% of patients), especially with ulcerative colitis. Early and high incidence of cholangiocarcinoma. 10% of patients with primary sclerosing cholangitis will develop cholangiocarcinoma. Page 9 of 38

10 MR cholangiopancreatography: -Multifocal strictures. -Segmental ectasias. -Ductal wall thickening. -Irregular beading of the bile ducts. Differential diagnostic with cholangiocarcinoma (periductal infiltrating type) is difficult when the disease is focal, manifesting as a focal short-segment stricture. Imaging findings CT and MR suggestive of development cholangiocarcinoma: -Focal mass. -Bile duct wall thickening greater than 4 mm. -Progressive biliary obstruction. II. RECURRENT PYOGENIC CHOLANGITIS (Figures 13 and 14) Secundary sclerosing cholangitis characterized by recurrent attacks of acute pyogenic cholangitis that occur in the setting of biliary obstruction by pigmented stones or biliary strictures. Fluke liver infestation (Ascaris lumbricoide, Clornochis sinensis) has been implicated as pathogens that may lead to initial cholangyocyte injury. Chronic, recurrent infections predispose to development of pigmented calculi, cholangitic abscesses and inflamatory strictures. Imaging manifestations: Page 10 of 38

11 -Biliary strictures. -Ductal wall thickening and enhancement secondary to fibrosis. -Intraductal pigmented stones. -Segmental distribution, predilection for the lateral segment of the left lobe. There is an increased risk (2-6%) of development of cholangiocarcinoma. The ductal wall thickening and enhancement may not be distinguished from cholangiocarcinoma (periductal infiltrating type) with imaging studies alone. III. MIRIZZI SYNDROME (Figures 15, 16 and 17)* Common hepatic duct obstruction secondary to compression by a gallstone impacted at the gallbladder neck or cystic duct. A low insertion of the cystic duct into the common hepatic duct is a predisposing factor. Imaging manifestations: -Extrinsic narrowing of the common hepatic duct. -A gallstone in the cystic duct. -Dilatation of the intrahepatic and common hepatic ducts. -Normal common bile duct. Inflammatory changes adjacent to the common bile duct and subsequent stricture may raise doubts with cholangiocarcinoma. Page 11 of 38

12 *The final pathological diagnosis was mucinous adenocarcinoma of the gallbladder. IV. HEPATOCELLULAR CARCINOMA (Figures 18 and 19) Hepatocellular carcinoma is the most common primary malignant hepatic neoplasm. Commonly occurs in a cirrhotic liver. Typical imaging appearance shows contrast enhancement in the arterial-phase and a washout of the lesion during the portal-venous phase. Some of them are hypovascular and best seen at portal-venous phase. A small cholangiocarcinoma may manifest as a hypervasular tumor, whereas most of the large mass-forming tumors manifest as a hypovascular mass with a hypervascular rim. Large lesions (>5cm) are heterogeneous with characteristic findings such as a mosaic pattern, tumoral capsule, necrosis and fatty metamorphosis. Hepatocellular carcinoma rarely show biliary ductal growth or invasion. Intrabiliary growth pattern appears as expansible soft-tissue masses that causes dilatation of the bile ducts. In this case may be difficult to distinguish from intrahepatic cholangiocarcinoma. V. BILIARY STONES (Figures 20, 21, 22, 23 and 24) Hepatolithiasis are a common cause of cholangiocarcinoma in endemic areas. Page 12 of 38

13 Intraductal cholangiocarcinoma may be confused with an intraductal masslike stone. Intraductal masslike stone shows absence of contrast enhancement and high attenuation at precontrast CT. An enhancing polypoid mass with asymmetric adjacent bile duct wall thickening is suggestive of an intraductal tumor. However, the presence of hepatolithiasis does not exclude the coexistence of an intraductal tumor. Images for this section: Page 13 of 38

14 Fig. 1: Mass-forming cholangiocarcinoma. (a) Arterial phase CT scan shows a homogeneous hypointense tumor of the liver with a rim enhancement at the periphery (arrow). Fig. 2: Mass-forming cholangiocarcinoma. (b) Delayed phase CT scan shows gradual centripetal enhancement of the hepatic tumor (arrow). Page 14 of 38

15 Fig. 3: Mass-forming cholangiocarcinoma. (c) Contrast-enhanced arterial phase MR image shows a low-signal-intensity mass in the right hepatic lobe, with irregular peripheral enhancement (arrow). Page 15 of 38

16 Fig. 4: Mass-forming cholangiocarcinoma. (d) Contrast-enhanced delayed phase MR image shows a gradual centripetal enhancement hepatic mass (arrow). Page 16 of 38

17 Fig. 5: Periductal infiltrating cholangiocarcinoma. (a) Hepatic venous phase CT scan shows dilated intrahepatic and hilar ducts (arrows). Page 17 of 38

18 Fig. 6: Periductal infiltrating cholangiocarcinoma. (b) Hepatic venous phase curve reconstruction CT scan shows a diffuse periductal thickening and increased enhancement in common hepatic duct, common bile duct and cystic duct (arrows). It also can be seen peripheral intrahepatic ductal dilatation (arrowhead). Page 18 of 38

19 Fig. 7: Intraductal cholangiocardinoma. (a) Arterial phase CT scan shows an intraductal polypoid mass (arrow) with focal ductal dilatation in the left hepatic lobe (arrowhead). Page 19 of 38

20 Fig. 8: Intraductal cholangiocardinoma. (b) Delayed phase CT scan shows an enhancing intraductal polypoid mass (arrow) with a localized dilatation in the left hepatic lobe (arrowhead). Page 20 of 38

21 Fig. 9: Intraductal cholangiocardinoma. (c) Contrast-enhanced arterial phase MR image shows ductal dilatation in the left hepatic lobe (arrowhead) with visible intraductal mass (arrow). Page 21 of 38

22 Fig. 10: Intraductal cholangiocardinoma. (d) Contrast-enhanced delayed phase MR image shows enhancing intraductal mass (arrow) with localized ductal dilatation in the left hepatic lobe (arrowhead). Page 22 of 38

23 Fig. 11: Primary sclerosing cholangitis. (a) Axial T2-weighted MR image shows segmental biliary dilatation in the hepatic lobes (arrows) and diffuse hypointense areas (*) of hepatic fibrosis (biliary cirrhosis). Page 23 of 38

24 Fig. 12: Primary sclerosing cholangitis. (b) Coronal image 3D T2-weighted MR Cholangiopancreatography image shows multifocal strictures (arrows) and segmental ectasias (arrowheads) and diffuse hypointense areas (*) of hepatic fibrosis (biliary cirrhosis). Page 24 of 38

25 Fig. 13: Recurrent pyogenic cholangitis. (a) 3D T2-weighted MR cholangiopancreatography image shows left hepatic duct stricture (arrow) and left peripheral intrahepatic ducts dilatation (arrowhead). Page 25 of 38

26 Fig. 14: Recurrent pyogenic cholangitis. (b) Axial SPAIR MR image shows left hepatic lobe atrophy and left intrahepatic bile ducts dilatation (arrow). Page 26 of 38

27 Fig. 15: Mirizzi syndrome. (a) Hepatic venous phase CT scan shows distended gallbladder (*) and intrahepatic biliary dilatation (arrow). Page 27 of 38

28 Fig. 16: Mirizzi syndrome. (b) SPAIR T2-weighted MR image shows a gallstone at the gallbladder neck (arrow) and intrahepatic bile ducts dilatation (arrowhead). Page 28 of 38

29 Fig. 17: Mirizzi syndrome. (c) SPAIR T2-weighted MR image shows normal common bile duct (arrow) and intrahepatic biliary dilatation (arrowhead). Page 29 of 38

30 Fig. 18: Hepatocellular carcinoma. (a) Arterial phase CT scan shows an heterogeneous intrahepatic mass with peripheral enhancement (arrow). Page 30 of 38

31 Fig. 19: Hepatocellular carcinoma. (b) Delayed phase CT scan shows a washout the lesion (arrow). Page 31 of 38

32 Fig. 20: Biliary stones I. (a) Precontrast phase curve reconstruction CT scan shows intrahepatic (arrows) and extrahepatic bile ducts dilatation (arrowhead). Page 32 of 38

33 Fig. 21: Biliary stones I. (b) Portal-venous phase curve reconstruction CT scan shows intrahepatic (arrows) and extrahepatic bile ducts dilatation (arrowhead). Page 33 of 38

34 Fig. 22: Biliary stones I. (c) 3D T2-weighted MR cholangiopancreatography image shows a impacted stone in distal common bile duct (arrowhead) with bile ducts dilatation (arrows). Page 34 of 38

35 Fig. 23: Biliary stones II. (a) Hepatic venous phase CT scan shows marked intrahepatic bile ducts dilatation (arrow). Page 35 of 38

36 Fig. 24: Biliary stones II. (b) SPAIR T2-Weighted MR image shows hypointense intraductal masslike stones (arrows). Page 36 of 38

37 Conclusion Cholangiocarcinoma is a malignant tumor arising from the biliary tract with a poor prognosis. There is a broad spectrum of inflammatory and neoplastic disorders of the intrahepatic and extrahepatic biliary system that resemble cholangiocarcinoma. It is essential to know the characteristic imagin findings of cholangiocarcinoma and the diverse entities that can mimic it, in order to perform an accurate diagnosis and appropriate treatment. Personal Information References 1. Menias CO, Surabhi VR, Prasad S.R., Wang HL, Narra VR, Chintapalli, KN Mimics of Cholangiocarcinoma : Spectrum of Disease.RadioGraphics 2008; 28: Chung YE, Kim MJ, Park YN, Choi JY, Pyo JY, Kim YC, Cho HJ, Kim KA, Choi SY Varying Appearances of Cholangiocarcinoma: Radiologic-Pathologic Correlation.RadioGraphics; 29: Lee WJ, Lim HK, Jang KM, et al. Radiologic spectrum of cholangiocarcinoma: emphasis on unusual manifestations and differential diagnoses. RadioGraphics 2001; 21: S97-S Vitellas KM, Keogan MT, Freed KS, et al. Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. RadioGraphics 2000; 20: BaronRL, Peterson ML,. From the RSN Refresher Courses Screening the Cirrhotic Liver for Hepatocellular Carcinoma with CT and MR Imaging: Opportunities and Pitfalls. RadioGraphics 2001; 21:S117-S132. Page 37 of 38

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