Korean J Gastroenterol.  2012 Aug;60(2):123-127. 10.4166/kjg.2012.60.2.123.

A Case of Intrahepatic Cholangiocarcinoma Associated with Type IV Choledochal Cyst

• Kim SH
• Kim HW
• Kang DH
• Kim MD
• Lee JH
• Lee JH
• Kim BG
• Park JH

• KMID: 1387342
• DOI: http://doi.org/10.4166/kjg.2012.60.2.123

Abstract

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.

Keyword

Anomalous union of pancreaticobiliary duct; Choledochal cyst; Intrahepatic cholangiocarcinoma

MeSH Terms

Adult
Bile Duct Neoplasms/*diagnosis/etiology/pathology
*Bile Ducts, Intrahepatic
Cholangiocarcinoma/*diagnosis/etiology/pathology
Cholangiopancreatography, Endoscopic Retrograde
Cholangiopancreatography, Magnetic Resonance
Choledochal Cyst/complications/*diagnosis
Female
Humans
Positron-Emission Tomography and Computed Tomography
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Abdominal CT findings. Abdominal CT scan showed papillary projected mass (arrow) with ductal dilation in the left intrahepatic duct (A) and ductal dilation of the right intrahepatic duct and the common bile duct (B), right intrahepatic solid mass (arrow) (C). Also abdominal CT showed both kidneys with large, multiple cysts.

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  Fig. 2 MRCP findings. (A) MRCP showed dilation of the common bile duct and anomalous union of pancreaticobiliary duct (arrow). (B) The additional small, papillary mass (white arrow) was also seen in the right intrahepatic duct (IHD) with large projected solid mass (black arrow) in left IHD.

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  Fig. 3 ERCP findings. (A) It showed fusiform dilation of the common bile duct and papillary mass in the left intrahepatic duct (IHD) (arrow). (B) Endoscopic naso biliary drainage tubography showed small, papillary mass in the right IHD (arrow).

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  Fig. 4 PET-CT findings. It showed hypermetabolic mass (SUVmax 14.9) in the left intrahepatic duct, suggesting malignant mass.

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  Fig. 5 Moderately differentiated adenocarcinoma was seen (H&E). (A) ×100, (B) ×400.

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