Background Despite an increasing number of long-term survivors after the resection of perihilar cholangiocarcinoma, metachronous carcinoma in the remnant distal bile duct has not been well documented because of its rarity. The aim of this study was to clarify the feasibility of operative resection and the pathologic features for metachronous double cholangiocarcinomas. Methods Between 2003 and 2013, 6 patients underwent resections for both a primary perihilar cholangiocarcinoma and a metachronous distal cholangiocarcinoma. Their medical records were retrospectively reviewed. Results At a median of 42 months (range, 19–138 months) after the hepatectomy, a metachronous distal cholangiocarcinoma was detected by follow-up computed tomography and, interestingly, no symptoms were observed. Despite severe adhesions, a pancreatoduodenectomy was undertaken in all patients; there were no serious complications, and the procedure resulted in an R0 resection. Although 2 patients died of the disease after the second operation, the remaining 4 patients are now alive with (n = 1) or without recurrence. A pathologic survey showed that 4 patients had changes of biliary intraepithelial neoplasia–2/3 around their primary and metachronous lesions. The primary and metachronous cholangiocarcinomas showed histologic similarity in 4 of the 6 patients and immunohistochemical concordance in 3 of the 6 patients. Conclusion Pancreatoduodenectomy for metachronous distal cholangiocarcinoma can lead to a favorable prognosis. Careful observation after the resection of perihilar cholangiocarcinoma is mandatory to detect this potentially curable disease. Pathologically, some of the multicentric cholangiocarcinomas present histologic and immunohistochemical similarities.
