title: Metastatic angiosarcomas: doxorubicin-based regimens, weekly paclitaxel and metastasectomy significantly improve the outcome.
abstract: BACKGROUND
  Angiosarcomas are a rare but aggressive form of soft tissue sarcoma. At metastatic stage, the clinical benefit of therapeutic intervention remains debatable.
  
  
  METHODS
  We have carried a retrospective analysis of 149 cases treated between 1996 and 2009 in the French Sarcoma Group.
  
  
  RESULTS
  The median age was 60; the sex ratio was 0.80. Sixty-two percentage of cases presented with metastasis at the diagnosis. About 20% arose in irradiated fields. The median overall survival was 11 months. Treatment consisted in metastasectomy (5.4%), doxorubicin-based regimen (46.9%), weekly paclitaxel (Taxol) (31.5%), other chemotherapy regimens (10.7%) or exclusive palliative care (10.9%). Clinical prognostic factors identified by univariate analysis were presence of bone metastasis (P = 0.0107), presence of other metastasis (P = 0.0327) and performance status (P < 0.0001). The Cox model retained a performance status of two or more as the sole independent prognostic factor (HR [hazard ratio] = 2.49, P < 0.0001). After adjustment to the performance status and compared with exclusive palliative care, the following treatments significantly improve the outcome: doxorubicin-based regimen as first-line chemotherapy (HR = 0.38, P = 0.0165), weekly paclitaxel as first-line regimen (HR = 0.36, P = 0.0146) and metastasectomy (HR = 0.09, P = 0.0221).
  
  
  CONCLUSIONS
  This retrospective analysis indicates that some therapeutic interventions may significantly improve the outcome of this aggressive disease. Doxorubicin-based regimens and weekly paclitaxel seem to provide the same range of efficacy.
authors.affiliation: Sarcoma Unit, Department of General Oncology, Centre Oscar Lambret, Lille, France. Mailn-penel@o-lambret.fr
authors.forename: N
authors.initials: N
authors.lastname: Penel
authors.forename: A
authors.initials: A
authors.lastname: Italiano
authors.forename: I
authors.initials: I
authors.lastname: Ray-Coquard
authors.forename: L
authors.initials: L
authors.lastname: Chaigneau
authors.forename: C
authors.initials: C
authors.lastname: Delcambre
authors.forename: Y M
authors.initials: YM
authors.lastname: Robin
authors.forename: B
authors.initials: B
authors.lastname: Bui
authors.forename: F
authors.initials: F
authors.lastname: Bertucci
authors.forename: N
authors.initials: N
authors.lastname: Isambert
authors.forename: D
authors.initials: D
authors.lastname: Cupissol
authors.forename: E
authors.initials: E
authors.lastname: Bompas
authors.forename: J O
authors.initials: JO
authors.lastname: Bay
authors.forename: F
authors.initials: F
authors.lastname: Duffaud
authors.forename: C
authors.initials: C
authors.lastname: Guillemet
authors.forename: J Y
authors.initials: JY
authors.lastname: Blay
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D002648:Child; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006394:Hemangiosarcoma / Q000188:drug therapy* / Q000556:secondary / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D017239:Paclitaxel / Q000008:administration & dosage; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D004317:Doxorubicin; D017239:Paclitaxel