title: Treatment and outcome of 82 patients with angiosarcoma.
abstract: BACKGROUND
  Angiosarcomas are an uncommon type of malignancy that are generally thought to behave usually in a locally aggressive fashion; they often metastasize to distant sites.
  
  
  METHODS
  Patients with a diagnosis of angiosarcoma treated at our institution between 1980 and 2006 were analyzed for patient demographics, tumor characteristics, multimodality treatment, and outcomes.
  
  
  RESULTS
  A total of 82 patients were divided into those with primary and advanced disease. Overall, the median age was 65 (range, 22-91) years, and 44% of patients were women. Median size of tumors was 3.8 cm, and 76% of tumors were intermediate or high grade. Tumors were located throughout the body: 32 cutaneous, 22 deep soft tissues or organs, 10 radiation or lymphedema field, 8 bone, and 7 nonirradiated breast. Of 46 patients with primary disease, all patients underwent surgical resection, 67% received radiotherapy, and 27% received chemotherapy. Five-year disease-specific survival was 60%, and negative prognostic factors included intermediate or high grade, and tumors arising in a radiated or lymphedema field. Of 36 patients with advanced disease, 36% underwent a palliative operation, 78% received radiation, and 58% received chemotherapy. Median survival was just 7.3 months, and cutaneous tumors predicted a better prognosis compared with other sites.
  
  
  CONCLUSIONS
  Primary angiosarcomas treated with aggressive surgical resection and the addition of radiation for close margins or worrisome pathologic features can result in long-term survival in most patients. The role of adjuvant chemotherapy is unclear. Patients with advanced disease have a poor prognosis, but there can be dramatic responses to chemotherapy in a minority of patients.
authors.affiliation: Orthopedic Oncology, Department of Orthopedics, Massachusetts General Hospital, Yawkey 7B-7926, 55 Fruit Street, Boston, Massachusetts 02114, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Abraham
authors.forename: Francis J
authors.initials: FJ
authors.lastname: Hornicek
authors.forename: Adam M
authors.initials: AM
authors.lastname: Kaufman
authors.forename: David C
authors.initials: DC
authors.lastname: Harmon
authors.forename: Dempsey S
authors.initials: DS
authors.lastname: Springfield
authors.forename: Kevin A
authors.initials: KA
authors.lastname: Raskin
authors.forename: Henry J
authors.initials: HJ
authors.lastname: Mankin
authors.forename: David G
authors.initials: DG
authors.lastname: Kirsch
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Rosenberg
authors.forename: G Petur
authors.initials: GP
authors.lastname: Nielsen
authors.forename: Vikram
authors.initials: V
authors.lastname: Desphpande
authors.forename: Herman D
authors.initials: HD
authors.lastname: Suit
authors.forename: Thomas F
authors.initials: TF
authors.lastname: DeLaney
authors.forename: Sam S
authors.initials: SS
authors.lastname: Yoon
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000473:pathology / Q000601:surgery; D001943:Breast Neoplasms / Q000473:pathology / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006394:Hemangiosarcoma / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D009367:Neoplasm Staging; D009381:Neoplasms, Radiation-Induced / Q000473:pathology / Q000601:surgery; D010166:Palliative Care; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000601:surgery; D015996:Survival Rate; D016896:Treatment Outcome