Dilatation of the intrahepatic bile duct of the left lateral segment of the liver at a medical checkup was pointed out in a woman in her 30s, but no solid lesion was observed. On her next annual medical checkup, the bile duct dilatation extended to the entire left lobe and the extrahepatic bile duct was also dilated. A 5-cm tumor was observed in the left lateral segment of the liver. After evaluation, we diagnosed intraductal papillary neoplasm of bile duct (IPN-B) and performed surgery. We performed extended left lobectomy, Spiegel lobectomy and biliary tract reconstruction. The tumor was a 5.5-cm multilocular cystic tumor and was definitively diagnosed as a mucin-producing intraductal papillary adenocarcinoma pathologically. She has no evidence of recurrence 11 months after surgery. IPN-B is known as a high risk tumor but its prognosis after surgery is good. The progression of the IPN-B is still unknown. In this IPN-B case, the tumor grew rapidly in one year but curative resection was possible.
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