The poor prognosis of patients with
intrahepatic cholangiocarcinoma (ICC) or
hilar cholangiocarcinoma is well known. Herein, we described the first reported case of severe locally advanced ICC in which radical surgery was successfully achieved based on the marked effect of
neoadjuvant chemoradiation therapy (NCRT) using
gemcitabine. A 54-year-old man was admitted to our institution with
obstructive jaundice. Abdominal computed tomography (CT) showed a large low-density mass in the caudate lobe, extensively involving the inferior vena cava and main portal vein. Moreover, nodal involvements of the hepatoduodenal ligament were detected concurrently. We therefore regarded this
tumor as a severe locally advanced ICC and attempted to initiate combined treatment with
gemcitabine (800 mg/m(2) biweekly) and three-dimensional conformation radiation (45 Gy/25 days). After completion of NCRT, this patient underwent a left trisegmentectomy with combined resection of the portal vein and inferior vena cava. Postoperative microscopic findings surprisingly revealed that more than 90% of
tumor cells had disappeared with extensive
fibrosis, achieving
tumor downstaging and
tumor volume reduction which were related to the radical resection. In conclusion, ICC showed a favorable histological response to chemoradiation
therapy using
gemcitabine. Further studies are needed to conclusively assess the effect of NCRT on locally advanced ICC.