We report a patient with inoperable hilar cholangiocarcinoma due to invasion at the umbilical portion who survived more than 4 years after right portal vein embolization and administration of S-1(50 mg/day). A 64-year-old male patient was immediately hospitalized for liver dysfunction and a high level of HbA1c. The disease was diagnosed as hilar cholangiocarcinoma mainly extending along the right hepatic duct. We made a request for operation to Nagoya University. He received right portal vein embolization in order to grow the residual liver but was deemed inoperable because of invasion at the umbilical portion. He refused chemotherapy but accepted administration of S-1(50 mg/day). Approximately 3 months after starting S-1, his ALP level normalized and about 9 months later stenting tube was lost. Subsequently, he returned to his job. Approximately 2 years and 2 months later, administration of S-1 was interrupted due to a harmful side effect. After approximately 13 months without S-1, the levels of CA19-9 and ALP again became elevated and administration of S-1 was restarted. He was temporarily hospitalized for abdominal pain and fever, but quickly recovered. Although CA19-9 and ALP levels re-normalized, he died after returning home. We emphasize the possibility of maintaining long-term health by minimal- dose S-1 therapy for inoperable hilar cholangiocarcinoma.

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