Background. Perihilar cholangiocarcinoma (PCC), defined as tumors involving or in close vicinity to the main bile duct confluence, is the most frequent type of cholangiocarcinoma (CC). Nevertheless, PCC occurring on biliary anatomical variant is a rare condition, difficult to diagnose and to establish the proper surgical approach.
Case presentation. A 59-year old male, with normal liver function, is incidentally diagnosed with PCC of the left hepatic duct (LHD), associated with a biliary anatomical variant consisting in right posterior hepatic duct (RPHD) entering the LHD. The patient underwent left hemihepatectomy with en-bloc resection of segment 1 and hilar lymph node dissection, and cholangiojejunostomy for the RPHD stump.
Results. The intraoperative and postoperative outcome were remarkably uneventful. Negative resection margins of the operative specimen were observed at pathological examination. No local recurrence occurred during a 2-yr follow-up.
Conclusion. Careful preoperative assessment and identification of anatomical variants are often the key to a successful surgical treatment in PCC.
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