Background: Sarcomatous intrahepatic cholangiocarcinoma (ICC) is a rare histological variant of ICC. The prognosis of sarcomatous ICC is poorly understood.
Methods: We analyzed the prognosis of sarcomatous ICC by reviewing the previous reports and our own case.
Results: Only 15 cases of sarcomatous ICC have been reported in the English-language literature so far. Median survival time of patients with sarcomatous ICC with and without surgery was 11 and 3 months, respectively. Survival rate of patients operated on for sarcomatous ICC was similar to that of patients with ordinary ICC without surgery in the early postoperative period. In the long-term view, however, the prognosis for the patients with sarcomatous ICC receiving surgery was better than that for the patients with ordinary ICC without surgery.
Conclusion: Although the prognosis for the patients with sarcomatous ICC was poor even after curative resection, surgery would be justified as the primary treatment for sarcomatous ICC.