Introduction Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma characterized by intraductal growth. There are few case reports and papers discussing treatment policy and prognosis for cases of postoperative relapse. Case Presentation The case subject was a female in her 40s. Due to IPNB with the primary tumor in hepatic left lobe, the patient underwent hepatic left lobe, caudate lobe, and bile duct resection, and biliary tract reconstruction at another institution. Four years later, near the cholangiojejunostomy, a localized recurrence was indicated. Though systemic metastasis was examined with CT, FDG-PET, distant metastasis was not confirmed. As it was believed that resection of the recurrent focal could lead to a long-term survival, the recurrent focal was resected. The resected specimen was a cystic mass with a film, and in a pathologic examination, lymph node tissue was not confirmed in the specimen, while intracystic papilloma of attached liver tissue without continuity was confirmed. The patient has not seen a relapse for 5 years and 5 months after the second surgery. Conclusion In a case of localized recurrence after the IPNB surgery, resection should be actively considered.
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