Management of primary liver sarcomas Journal Article

Authors: Weitz, J.; Klimstra, D. S.; Cymes, K.; Jarnagin, W. R.; D'Angelica, M.; La Quaglia, M. P.; Fong, Y.; Brennan, M. F.; Blumgart, L. H.; DeMatteo, R. P.
Article 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 RO-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 RO-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 RO-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. © 2007 American Cancer Society.
Keywords: adolescent; adult; cancer survival; child; clinical article; preschool child; school child; treatment outcome; aged; aged, 80 and over; middle aged; survival rate; liver neoplasms; cancer patient; cancer grading; prospective studies; demography; antineoplastic combined chemotherapy protocols; angiosarcoma; hemangiosarcoma; sarcoma; liver; fibrosarcoma; tumor recurrence; surgery; hemangiopericytoma; leiomyosarcoma; solitary fibrous tumor; dying; therapy; rare disease; carcinosarcoma; hemangioendothelioma; hemangioendothelioma, epithelioid; embryonal sarcoma; liver histology; liver sarcoma
Journal Title: Cancer
Volume: 109
Issue: 7
ISSN: 0008-543X
Publisher: Wiley Blackwell  
Date Published: 2007-04-01
Start Page: 1391
End Page: 1396
Language: English
DOI: 10.1002/cncr.22530
PUBMED: 17315167
PROVIDER: scopus
Notes: --- - "Cited By (since 1996): 35" - "Export Date: 17 November 2011" - "CODEN: CANCA" - "Source: Scopus"
Altmetric Score
MSK Authors
  1. Jurgen Weitz
    8 Weitz
  2. Murray F Brennan
    755 Brennan
  3. Leslie H Blumgart
    339 Blumgart
  4. Ronald P DeMatteo
    596 DeMatteo
  5. Karina Cymes
    5 Cymes
  6. David S Klimstra
    843 Klimstra
  7. William R Jarnagin
    582 Jarnagin
  8. Yuman Fong
    740 Fong