Management of primary liver sarcomas

• Published in: Cancer 2007-04, Vol.109 (7), p.1391-1396
• Main Authors: Weitz, Juürgen, Klimstra, David S., Cymes, Karina, Jarnagin, William R., D'Angelica, Michael, La Quaglia, Michael P., Fong, Yuman, Brennan, Murray F., Blumgart, Leslie H., DeMatteo, Ronald P.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Carcinosarcoma - therapy; Child; Female; Fibrosarcoma - therapy; Hemangioendothelioma, Epithelioid - therapy; Hemangiosarcoma - therapy; Humans; Leiomyosarcoma - therapy; liver; Liver Neoplasms - drug therapy; Liver Neoplasms - radiotherapy; Liver Neoplasms - surgery; Liver Neoplasms - therapy; Male; Medical sciences; Middle Aged; Prognosis; Prospective Studies; sarcoma; Sarcoma - drug therapy; Sarcoma - radiotherapy; Sarcoma - surgery; Sarcoma - therapy; surgery; Survival Rate; therapy; Treatment Outcome; Tumors
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/cncr.22530

BACKGROUND Primary hepatic sarcoma is a rare entity. The objectives of the study were to define treatment and long‐term outcome and to identify prognostic factors. METHODS Between 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. RESULTS Of 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 R0‐resection and had a 5‐year disease‐specific survival of 64%. Of these 11 patients, 4 had low‐grade sarcoma and have not developed tumor recurrence. In the group of 7 patients with high‐grade sarcomas who underwent R0‐resection, both patients with angiosarcoma died within 11 months, whereas the 5 patients with embryonal sarcoma had a 5‐year disease‐free and disease‐specific 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 5‐year disease‐specific survival of 67%. Of the remaining 13 patients in whom R0‐resection was not performed, there were no 3‐year survivors. The prognosis for patients with primary carcinosarcoma of the liver was poor, with all but 1 patient dying within a year and no 3‐year survivors. CONCLUSIONS The 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. Primary hepatic sarcomas are rare tumors. The treatment and outcome of 30 patients with primary sarcoma and 5 patients with carcinosarcoma of the liver are reported.