Multicentric recurrence of intraductal papillary neoplasms of bile duct in the remnant intrahepatic bile duct after curative resection

• Intraductal papillary neoplasm of the bile duct (IPNB) is a newly-recognized disease concept and its long-term prognosis and pattern of recurrence are poorly understood so far.
• We report a case of IPNB patient with early stage carcinoma who had multicentric recurrence in the remnant hepatic bile duct around 2 years after R0 resection.
• We should bear in mind multicentric remnant intrahepatic bile duct recurrence in IPNB patients with multiple lesions.
• Endoscopic approach using double balloon enteroscopy is useful in diagnosis of recurrence and palliation of symptoms in selected patients.

IntroductionThere have been few reports on the prognosis of patients with intraductal papillary neoplasms of the bile duct (IPNB). Here we report a case of IPNB in a patient with early-stage carcinoma who had multicentric recurrence in the remnant hepatic bile duct after curative resection.Case presentationA 78-year-old man with hepatic dysfunction and cholestasis was referred to our hospital. Preoperative imaging studies revealed the presence of papillary tumors in the left hepatic duct and common hepatic duct, while no tumor lesions were detected in the right hepatic duct. This patient underwent left hepatectomy, extra-hepatic bile duct resection with biliary reconstruction, and regional lymphnode dissection. On the basis of pathological examination, this patient was diagnosed with multiple IPNB with early-stage adenocarcinoma with negative surgical margin. Postoperative work-up was periodically performed, indicating no evidence of recurrence, while the patient had sustained hepatic dysfunction, cholestasis, and repetitive cholangitis since the early postoperative period. Finally, recurrence in the remnant intrahepatic bile duct of the posterior segment was revealed by double balloon enteroscopy at 29 months after surgery. At 34 months after surgery, internal drainage stents were replaced in both endoscopic and percutaneous manners within the relapsed intrahepatic bile ducts to address repetitive cholangitis. These procedures enabled the patient to remain asymptomatic until death at 41 months after surgery.DiscussionMulticentric recurrence in the remnant intrahepatic bile duct after surgery may occur in IPNB patients with multiple lesions. An endoscopic approach may be useful in such cases, not only in the diagnosis of remnant intrahepatic bile duct recurrence but also for palliation of symptoms.