An 80-year-old woman visited our department with the complaint of icterus and brown urine. After detailed examination, she was diagnosed with cT4N0M (-), cStage IVa, hilar cholangiocarcinoma. We believed that she could be cured with surgery, but she and her family did not agree to the surgical procedure. Chemotherapy was scheduled, and gemcitabine (GEM) therapy was started in April 2011. GEM therapy reduced significantly the level of the CA 19-9 tumor marker, and diagnostic imaging allowed a judgment of partial response or stable disease after 18 months. In the present report, we describe a case of a patient with hilar cholangiocarcinoma who achieved long-term survival with GEM therapy. We also include a brief literature review.
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