title: Outcomes of Systemic Therapy for Patients with Metastatic Angiosarcoma.
abstract: BACKGROUND
  Angiosarcomas (AS) are rare tumors of vascular origin with a variable behavior and overall poor prognosis. We sought to assess the outcomes of patients treated for metastatic disease.
  
  
  METHODS
  We performed a retrospective analysis of 119 patients treated for metastatic AS. Outcomes and efficacy measurements of the first and subsequent lines of treatment were analyzed.
  
  
  RESULTS
  Median age was 61 years, and the most frequent primary sites were chest wall/breast (31%), viscera (22%) and head/neck (20%). Seventy-three (61%) and 46 (39%) patients received ≥ 2 and ≥ 3 lines of therapy, respectively. The most commonly used agents included taxanes and anthracyclines. Median overall survival was 12.1 months. Median times to tumor progression were 3.5 months for first line, 3.7 months for second line and 2.7 months for third line. Among 48 patients evaluable per RECIST, the overall response rate to first line was 30% and <10% in subsequent lines. Doxorubicin, liposomal doxorubicin and taxanes resulted in similar response rates and survival, and there was no apparent benefit for combination chemotherapy.
  
  
  CONCLUSIONS
  Despite reasonable response rates in the first-line setting, benefit from systemic therapy is short-lived in metastatic AS, and outcomes are poor. Doxorubicin, liposomal doxorubicin and taxanes are reasonable and appropriate choices for monotherapy.
authors.affiliation: Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, N.Y., USA.
authors.forename: Sandra P
authors.initials: SP
authors.lastname: D'Angelo
authors.forename: Rodrigo R
authors.initials: RR
authors.lastname: Munhoz
authors.forename: Deborah
authors.initials: D
authors.lastname: Kuk
authors.forename: Johnathan
authors.initials: J
authors.lastname: Landa
authors.forename: Eliza W
authors.initials: EW
authors.lastname: Hartley
authors.forename: Michael
authors.initials: M
authors.lastname: Bonafede
authors.forename: Mark A
authors.initials: MA
authors.lastname: Dickson
authors.forename: Mrinal
authors.initials: M
authors.lastname: Gounder
authors.forename: Mary L
authors.initials: ML
authors.lastname: Keohan
authors.forename: Aimee M
authors.initials: AM
authors.lastname: Crago
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.forename: William D
authors.initials: WD
authors.lastname: Tap
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018943:Anthracyclines / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D018450:Disease Progression; D004317:Doxorubicin / Q000031:analogs & derivatives / Q000627:therapeutic use; D005260:Female; D006394:Hemangiosarcoma / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011092:Polyethylene Glycols / Q000627:therapeutic use; D012189:Retrospective Studies; D043823:Taxoids / Q000627:therapeutic use; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D018943:Anthracyclines; D000970:Antineoplastic Agents; D043823:Taxoids; C506643:liposomal doxorubicin; D011092:Polyethylene Glycols; D004317:Doxorubicin