Korean J Radiol.  2002 Mar;3(1):57-63. 10.3348/kjr.2002.3.1.57.

Radiological Spectrum of Intraductal Papillary Tumors of the Bile Ducts

• Lim JH
• Yi CA
• Lim HK
• Lee WJ
• Lee SJ
• Kim SH

• KMID: 1758448
• DOI: http://doi.org/10.3348/kjr.2002.3.1.57

Abstract

Papillary tumor of the bile duct is characterized by the presence of an intraductal tumor with a papillary surface comprising innumerable frondlike infoldings of proliferated columnar epithelial cells surrounding slender fibrovascular stalks. There may be multiple tumors along the bile ducts (papillomatosis or papillary carcinomatosis), which are dilated due to obstruction by a tumor per se, by sloughed tumor debris, or by excessive mucin. Radiologically, the biliary tree is diffusely dilated, either in a lobar or segmental fashion, or aneurysmally, depending on the location of the tumor, the debris, and the amount of mucin production. A tumor can be depicted by imaging as an intraductal mass with a thickened and irregular bile duct wall. Sloughed tumor debris and mucin plugs should be differentiated from bile duct stones. Cystically or aneurysmally, dilated bile ducts in mucin-hypersecreting variants (intraductal papillary mucinous tumors) should be differentiated from cystadenoma, cystadenocarcinoma and liver abscess.

Keyword

Bile ducts, neoplasms; Bile ducts, CT; Bile ducts, US; Bile ducts, interventional procedures

MeSH Terms

Bile Duct Neoplasms/*radiography
*Bile Ducts, Extrahepatic
*Bile Ducts, Intrahepatic
Carcinoma, Papillary/radiography
Cystadenoma, Mucinous/radiography
Diagnosis, Differential
Female
Human
Male
Middle Age

Figure

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  Fig. 1 Schematic drawing of intraductal papillary tumors of the biliary tract (A: carcinoma; B: adenoma; C: dysplasia; m: mucus).

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  Fig. 2 Microphotograph of papillary carcinoma demonstrates papillae comprising a fibrovascular core and a covering of columnar epithelial cells. The term 'papillary' derives from the frondlike papillary infoldings (arrows) (H & E staining, ×100).

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  Fig. 3 A 64-year-old man with papillary carcinoma arising in the left intrahepatic bile duct. A. Transverse sonogram of the left lobe of the liver reveals a fairly well-defined oval echogenic mass measuring 1.5 cm in its lateral segment (arrows), as well as markedly dilated bile ducts. B. CT image shows an intraluminal mass (arrow) and dilated peripheral bile ducts. C. Photomicrograph of a gross specimen depicts intraductal papillary carcinoma. Note the presence of myriads of frondlike papillary infoldings into the lumen of the bile ducts (arrows) (H & E staining, ×10).

• 

  Fig. 4 A 63-year-old man with papillary adenomatosis in the common hepatic duct. A. Sonogram reveals dilated extrahepatic bile ducts and an echogenic cast filling the common hepatic duct (curved arrows). Note the presence of a slit-like defect in the cast (arrow). B. Post-contrast CT image shows the thickened wall of the extrahepatic bile duct (open arrow). Note that in the center of the duct, a slit-like lumen is present. C. Endoscopic retrograde cholangiogram depicts a papillary tumor in the common hepatic duct (arrows) and severe dilatation of the intra- and extrahepatic bile ducts. Note the velvety appearance, serrated margin, and nodular filling defects of the involved segment.

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  Fig. 5 A 56-year-old woman with papillary adenomatosis in which multifocal foci of carcinomatous change are seen in the gallbladder and cystic duct. A. Sonogram of the gallbladder reveals an echogenic mass with a ragged surface (arrows). B. Post-contrast CT image depicts an enhancing mass (arrows) in the gallbladder. C. Endoscopic retrograde cholangiogram indicates that the gallbladder mass has a nodular and velvety appearance (arrows). D. Photograph of resected gallbladder depicts an irregular mass, velvety in appearance, comprised of myriads of papillary projections.

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  Fig. 6 A 54-year-old woman with a mucin-producing, intraductal mucosal-spreading, papillary adenocarcinoma causing cystic and tubular dilatation of the intrahepatic bile ducts, complicated by abscess and rupture. (illustrations referred to in reference 7, and have been used with the authors' permission.) A. Contrast-enhanced CT scan of the left lobe obtained during the equilibrium phase shows balloon-like dilatation of the left lobar and segmental bile ducts (asterisk), as well as severe dilatation of surrounding peripheral bile ducts (arrows). B. CT scan 9 cm caudal to A demonstrates cyst-like (asterisk) and tubular dilatation of the bile ducts of the caudate lobe. Note that in the right hepatic lobe, the bile duct is slightly dilated. C. Endoscopic retrograde cholangiogram indicates that the extrahepatic bile ducts contain thread-and-streak-like multiple linear filling defects, representing mucus. The left hepatic duct is completely obstructed, and the right, partially so. In the right hepatic lobe, the bile duct is again slightly dilated. D. Photomicrograph of a resected specimen depicts balloon-like dilatation of the bile ducts, which are covered with diffusely thickened fibrotic wall. The luminal surface of one duct is lined by short papillary infoldings (star) but on other surfaces, papillary infoldings are denuded (asterisks). The arrow points to tumor growth at Glisson's capsule, due to perforation of the cystic tumor (H & E staining, ×10).

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  Fig. 7 A 59-year-old woman with papillary carcinomatosis in the left hepatic ducts and extrahepatic ducts. A. CT image of the liver during the equilibrium phase shows markedly dilated, tortuous, crowded bile ducts in the left hepatic lobe. The percutaneous transhepatic catheter present in the extrahepatic duct is displaced posteriorly due to the intraluminal mass (arrow). B. Percutaneous transhepatic cholangiogram demonstrates multiple nodular filling defects in the extrahepatic ducts (arrows), simulating multiple stones. Due to obstruction, the left hepatic duct is not opacified.

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  Fig. 8 A 62-year-old man with recurrent pyogenic cholangitis and resultant bile duct stricture. A. Sonogram of the right hepatic lobe reveals substantially dilated posterior segmental bile ducts. There is no visible mass or stone. B, C. Computed tomogram (B) and MR cholangiogram (C) depict dilated posterior segmental bile ducts with no identifiable mass or stone.

Reference

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6. Hubens G, Delvaux G, Willems G, Bourgain C, Kloppel G. Papillomatosis of the intra- and extrahepatic bile ducts with involvement of the pancreatic duct. Hepatogastroenterology. 1991. 38:413–418.

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