title: Management of primary liver sarcomas.
abstract: 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.
authors.affiliation: Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York, USA. dematter@mskcc.org
authors.forename: Juürgen
authors.initials: J
authors.lastname: Weitz
authors.forename: David S
authors.initials: DS
authors.lastname: Klimstra
authors.forename: Karina
authors.initials: K
authors.lastname: Cymes
authors.forename: William R
authors.initials: WR
authors.lastname: Jarnagin
authors.forename: Michael
authors.initials: M
authors.lastname: D'Angelica
authors.forename: Michael P
authors.initials: MP
authors.lastname: La Quaglia
authors.forename: Yuman
authors.initials: Y
authors.lastname: Fong
authors.forename: Murray F
authors.initials: MF
authors.lastname: Brennan
authors.forename: Leslie H
authors.initials: LH
authors.lastname: Blumgart
authors.forename: Ronald P
authors.initials: RP
authors.lastname: Dematteo
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002296:Carcinosarcoma / Q000628:therapy; D002648:Child; D005260:Female; D005354:Fibrosarcoma / Q000628:therapy; D018323:Hemangioendothelioma, Epithelioid / Q000628:therapy; D006394:Hemangiosarcoma / Q000628:therapy; D006801:Humans; D007890:Leiomyosarcoma / Q000628:therapy; D008113:Liver Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012509:Sarcoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome