Korean J Hepatobiliary Pancreat Surg.  2016 Aug;20(3):137-143. 10.14701/kjhbps.2016.20.3.137.

Surgical treatment of mucin-producing cholangiocarcinoma arising from intraductal papillary neoplasm of the intrahepatic bile duct: a report of 2 cases

• Baterdene
• Hwang S
• Lee JW
• Jung MJ
• Shin H
• Seo HK
• Kim MH
• Lee SK

• KMID: 2351302
• DOI: http://doi.org/10.14701/kjhbps.2016.20.3.137

Abstract

Intraductal papillary neoplasms of the bile duct (IPNB) leads to malignant transformation and mucin production. Herein, we presented two cases of mucin-producing IPNB with obstructive jaundice who underwent resection of the intrahepatic lesions and bypass hepaticojejunostomy. The first case was a 69 year-old male patient with 5-year follow up for gallstone disease. Imaging studies showed mucin-secreting IPNB mainly in the hepatic segment III bile duct (B3) and multiple intrahepatic duct stones for which, segment III resection, intrahepatic stone removal, end-to-side choledochojejunostomy and B3 hepaticojejunostomy were conducted. The second case was a 74 year-old female patient with 11-year follow up for gallstone disease. Imaging studies showed mucin-producing IPNB with dilatation of the segment IV duct (B4) and mural nodules for which, segment IV resection, partial resection of the diaphragm and central hepaticojejunostomy were conducted. Both patients recovered uneventfully from surgery. These cases highlight that in patients with IPNB, abundant production of highly viscous mucin inducing obstructive jaundice may be associated with malignant transformation.

Keyword

Intrahepatic cholangiocarcinoma; Papillary growth; Intraductal growth; Mucin production; Jaundice

MeSH Terms

Bile Ducts
Bile Ducts, Intrahepatic*
Cholangiocarcinoma*
Choledochostomy
Diaphragm
Dilatation
Female
Follow-Up Studies
Gallstones
Humans
Jaundice
Jaundice, Obstructive
Male
Mucins
Mucins

Figure

• 

  Fig. 1 Preoperative imaging study findings of Case 1. (A) Computed tomography image shows diffuse dilatation of the intrahepatic duct and stones; (B) Magnetic resonance cholangiography shows markedly dilated intrahepatic ducts filled with mucin and stones; (C) Endoscopic retrograde cholangiography shows filling defects at the intrahepatic ducts and common bile duct; and (D) Endoscopic retrograde cholangiography shows high-viscosity mucin drainage from the ampulla of Vater.

• 

  Fig. 2 Operative photographs of Case 1. (A) At laparotomy, the segment III cystic lesion is protruded; (B) The common bile duct is filled with mucin; (C) The segment III cystic lesion is dissected from the hepatic parenchyma; (D) The segment III cystic lesion is excised; (E) Endto-side choledochojejunostomy is performed; and (F) B3 hepaticojejunostomy is completed.

• 

  Fig. 3 Postoperative hepatobiliary scintigraphy of Case 1 showing uneventful biliary drainage.

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  Fig. 4 Preoperative imaging study findings of Case 2. (A) Computed tomography image shows a cystic dilatation at the segment IV; (B) Magnetic resonance cholangiography shows markedly dilated left intrahepatic ducts filled with mucin; (C) Percutaneous transhepatic biliary drainage shows filling defects at the left intrahepatic ducts and common bile duct; and (D) Percutaneous transhepatic cholangioscopy shows high-viscosity mucin within the left intrahepatic duct.

• 

  Fig. 5 Operative photographs of Case 2. (A) The common bile duct is filled with mucin; (B) The segment IV cystic lesion is excised; (C) Two large openings to the right and left hepatic ducts are unified with running sutures and then reconstructed with single large hepaticojejunostomy; and (D) Central hepaticojejunostomy is finished.

• 

  Fig. 6 Postoperative hepatobiliary scintigraphy of Case 2 showing uneventful biliary drainage.

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