先天性胆道拡張症に合併した肝内胆管原発小細胞癌の１例

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  • A CASE OF SMALL CELL CARCINOMA OF THE INTRAHEPATIC BILE DUCT WITH CONGENITAL BILIARY DILATATION

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A 33-year-old woman who had the abrupt onset of right upper abdominal pain during pregnancy visited a hospital on October 17, 2003. Following abdominal ultrasonographic study which disclosed dilatation of the intrahepatic bile duct, she was referred to our hospital for close exploration. Abdominal ultrasonography at our hospital revealed cystic dilatation of the common and intrahepatic bile ducts and an about 45mm-sized well-defined and solid tumor in the left intrahepatic bile duct. After other examinations bile duct cancer at the left porta hepatis associated with congenital dilatation of bile duct was suspected, and left lobectomy of liver and excision of the extrahepatic bile duct were performed on February 4, 2004 when she had been delivered. On the removed material we confirmed an elastic-soft and solid tumor, with a yellowish white section, projecting into the dilated left intrahepatic bile duct, and then diagnosed it as small cell carcinoma of the bile duct histopathologically. Abdominal CT scan performed one year after the operation showed recurrence at the surgical stump of liver. She has been followed under chemotherapy at present.<br> Small cell carcinoma of the bile duct is a rare entity and a total of 13 cases, including ours, have been reported, but only our case is of the intrahepatic origin. Many patients have poor prognosis even after radical surgery so that aggressive postoperative adjuvant therapies must be considered.

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