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Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst

      Abstract

      Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years. Computed tomographic scan and percutaneous transhepatic cholangioscope revealed multiple stones and stenosis of the hepatic duct and the left intrahepatic bile duct. Histologic examination of a biopsy specimen obtained from the stenotic site showed adenocarcinoma of the intrahepatic bile duct. Left lobectomy with re-reconstruction by right hepaticojejunostomy was performed, and his postoperative course was uneventful. One year after the operation, however, he died of carcinomatous peritonitis with recurrence of cholangiocarcinoma. This report warns us that bile stasis owing to stenosis of the intrahepatic bile duct and repeated cholangitis with multiple stones are high-risk factors for carcinogenesis of the intrahepatic bile duct even after total resection of the infantile choledochal cyst.

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      References

        • Ono S.
        • Tokiwa K.
        • Iwai N.
        Cellular activity in the gallbladder of children with anomalous arrangement of the pancreaticobiliary duct.
        J Pediatr Surg. 1999; 34: 962-966
        • Tokiwa K.
        • Iwai N.
        Early mucosal changes of the gallbladder in patients with anomalous arrangement of the pancreaticobiliary duct.
        Gastroenterology. 1996; 110: 1614-1618
        • Watanabe Y.
        • Toki A.
        • Todani T.
        Bile duct cancer developed after cyst excision for choledochal cyst.
        J Hepatobiliary Pancreat Surg. 1999; 6: 207-212
        • Kobayashi S.
        • Asano T.
        • Yamasaki M.
        • et al.
        Prophylactic excision of the gallbladder and bile duct for patients with pancreaticobiliary maljunction.
        Arch Surg. 2001; 136: 759-763
        • Iwai N.
        • Deguchi E.
        • Yanagihara J.
        • et al.
        Cancer arising in a choledochal cyst in a 12-year-old girl.
        J Pediatr Surg. 1990; 25: 1261-1263
        • Ueda J.
        • Neduka H.
        • Yamamoto S.
        • et al.
        A case of pancreaticobiliary maljunction associated with congenital bile duct dilatation with early bile duct cancer in a 13-year-old girl (in Japanese).
        Tan To Sui. 2000; 21: 593-597
        • Tokiwa K.
        • Ono S.
        • Iwai N.
        Mucosal cell proliferation activity of the gallbladder in children with anomalous arrangement of the pancreatobiliary duct.
        J Hepatibiliary Pancreat Surg. 1999; 6: 213-217
        • Iwai N.
        • Yanagihara J.
        • Tokiwa K.
        • et al.
        Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract.
        Ann Surg. 1992; 215: 27-30
        • Tohi T.
        • Morita M.
        • Ishibashi T.
        • et al.
        Complications after biliary reconstruction for choledochal cyst (in Japanese).
        Proc Jpn Study Group on Pancreaticobiliary Maljunction. 1998; 21: 96-97
        • Nakahara S.
        • Nishihara K.
        • Takeda N.
        • et al.
        A case of small cell carcinoma after cyst excision (in Japanese).
        Nippon Shokakigeka Gakkai Zasshi (Jpn J Gastroenterol Surg). 1996; 29: 531
        • Uno K.
        • Tsuchida Y.
        • Kawarazaki H.
        • et al.
        Development of intrahepatic cholelithiasis long after primary excision of choledochal cysts.
        J Am Coll Surg. 1996; 183: 583-588
        • Ando H.
        • Ito T.
        • Kaneko K.
        • et al.
        Congenital stenosis of the intrahepatic bile duct associated with choledochal cyst.
        J Am Coll Surg. 1995; 181: 426-430
        • Todani T.
        • Watanabe Y.
        • Urushihara N.
        • et al.
        Biliary complication after excisional procedure for choledochal cyst.
        J Pediatr Surg. 1995; 30: 478-481
        • Matsubara T.
        • Sakurai Y.
        • Sasayama Y.
        • et al.
        K-ras point mutations in cancerous and noncancerous biliary epithelium in patients with pancreaticobiliary maljunction.
        Cancer. 1996; 77: 1752-1757
        • Tanno S.
        • Obara T.
        • Fujii T.
        • et al.
        Proliferative potential and K-ras mutation in epithelial hyperplasia of the gallbladder in patients with anomalous pancreaticobiliary ductal union.
        Cancer. 1998; 83: 267-275
        • Hanada K.
        • Itoh M.
        • Fujii K.
        • et al.
        K-ras and p53 mutation in stage I gallbladder carcinoma with an anomalous junction of the pancreaticobiliary duct.
        Cancer. 1996; 77: 452-458
        • Iwase T.
        • Nakazawa S.
        • Yamao K.
        • et al.
        Ras gene point mutation in gallbladder lesions associated with anomalous connection of pancreatobiliary ducts.
        Hepatogastroenterology. 1997; 44: 1457-1462