BACKGROUNDPrimary hepatic sarcoma is a rare entity. The objectives of the study were to define treatment and longâterm outcome and to identify prognostic factors.METHODSBetween January 1981 and December 2004, 30 patients with primary sarcoma of the liver and 5 patients with primary carcinosarcoma of the liver were treated. Patient demographics, tumor characteristics, treatment, and actuarial survival were analyzed.RESULTSOf the 30 patients with primary hepatic sarcoma (10 epithelioid hemangioendothelioma, 5 embryonal sarcoma, 5 angiosarcoma, 3 solitary fibrous tumor, and 7 other types), 11 underwent R0âresection and had a 5âyear diseaseâspecific survival of 64%. Of these 11 patients, 4 had lowâgrade sarcoma and have not developed tumor recurrence. In the group of 7 patients with highâgrade sarcomas who underwent R0âresection, both patients with angiosarcoma died within 11 months, whereas the 5 patients with embryonal sarcoma had a 5âyear diseaseâfree and diseaseâspecific survival of 80%. Six of the 10 patients with an epithelioid hemangioendothelioma were managed without surgery, as they had diffuse, slowly progressing, or stable lesions; these patients had a 5âyear diseaseâspecific survival of 67%. Of the remaining 13 patients in whom R0âresection was not performed, there were no 3âyear survivors. The prognosis for patients with primary carcinosarcoma of the liver was poor, with all but 1 patient dying within a year and no 3âyear survivors.CONCLUSIONSThe outcome of patients with primary hepatic sarcoma depends primarily on tumor histology and the ability to achieve complete tumor resection. Improvements in outcome will require the development of more effective systemic therapies. Cancer 2007. © 2007 American Cancer Society.