Intraductal papillary neoplasm of the bile duct with rapidly progressive multicentric recurrence

INTRODUCTION: Knowledge on the pattern of recurrence and prognosis of intraductal papillary neoplasms of the bile duct (IPNB) is limited. Few studies have reported IPNB recurrence in the remnant intrahepatic bile duct, which is indicative of the true multicentricity of IPNB. Herein, we report a case of IPNB with rapidly progressive recurrence in the remnant intrahepatic bile duct and review the literature for discussing the prognosis of IPNB with multicentricity. CASE PRESENTATION: A 72-year-old male was diagnosed with IPNB in the hepatic duct of segment 3 that had spread to the left hepatic duct. The patient underwent left hepatectomy, total caudate lobectomy, and extra-hepatic bile duct resection with biliary reconstruction. Histologically, the tumor was IPNB with noninvasive adenocarcinoma with a negative surgical margin. Although dilatation of B8 and biliary enzyme elevation were observed beginning at 7-10 months postoperatively, there was no evidence of recurrence. At 17 months postoperatively, the recurrent tumor diffusely spread throughout the remnant intrahepatic bile duct. Internal drainage stents were placed within the intrahepatic bile ducts with relapsed IPNB to relieve jaundice, and a course of chemotherapy was considered. However, the patient did not receive any therapies up to his death at 21 months postoperatively because of rapid disease progression. CONCLUSION: According to a literature review, some cases of multicentric IPNB have shown rapidly progressive recurrence and poor prognosis. We should consider multicentricity of IPNB even a few months after curative resection, and narrow examinations should also be considered.