RESUMEN
A 39-year-old female patient presenting with epigastric discomfort and jaundice was diagnosed as diffuse hilar cholangiocarcinoma with vascular tumor invasion. After preoperative management with percutaneous transhepatic biliary drainage and right portal vein embolization, the patient underwent modified hepatoligamentopancreatoduodectomy; this included extended right hepatectomy, caudate lobectomy, portal vein segmental resection, enbloc resection of hepatoduodenal ligament together with proper hepatic artery resection and with leaving a replaced left hepatic artery from the left gastric artery, and paraaortic lymph node dissection. Intraoperative frozen histologic examination revealed multiple paraaortic lymph node metastases, which was generally regarded as definite distant metastasis. There was no postoperative complication except for persistent drainage of ascites that occurred only during the immediate postoperative period. She did not receive postoperative adjuvant chemotherapy or radiotherapy. The patient is now living well 11 years 2months after surgery without recurrence. Although this patient revealed hilar vascular invasion and paraaortic lymph node metastasis, she survived more than 10 years without tumor recurrence. This implicate that aggressive surgical treatment of hilar cholangiocarcinoma can result in long-term survival even in the presence of paraaortic lymph node metastasis.