title: Treatment and prognostic factors of radiation-associated angiosarcoma (RAAS) after primary breast cancer: a systematic review.
abstract: BACKGROUND
  Radiation-associated angiosarcoma (RAAS) of the breast is a rare, aggressive disease. The incidence is increasing with the prolonged survival of women irradiated for primary breast cancer. Surgery is the current treatment of choice. Prognosis is poor. This review aims to evaluate all publications on primary treatment of RAAS to identify prognostic factors and evaluate treatment modalities.
  
  
  METHODS
  Databases were searched for articles with published individual patient data on prognostic factors, treatment and follow-up of patients with RAAS. A regression analysis was performed to test the prognostic values of age, interval between primary treatment and RAAS, tumour size and grade on the local recurrence-free interval (LRFI) and overall survival (OS). The effects of treatment modalities surgery, radiation (with or without hyperthermia) and chemotherapy or combinations were evaluated.
  
  
  RESULTS
  74 articles were included, representing data on 222 patients. In these patients, the 5-year OS was 43% and 5-year LRFI was 32%. Tumour size and age were significant prognostic factors on LRFI and OS. Of all patients, 68% received surgery alone, 17% surgery and reirradiation and 6% surgery with chemotherapy. The remaining 9% received primary treatments without surgery. Surgery with radiotherapy had a better 5-year LRFI of 57% compared to 34% for surgery alone (p=0.008). The value of other treatment modalities could not be assessed.
  
  
  CONCLUSIONS
  This systematic review confirms the poor prognosis of RAAS. Tumour size and age were of prognostic value. The addition of reirradiation to surgery in the treatment of RAAS appears to enhance local control.
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: A L
authors.initials: AL
authors.lastname: Depla
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: C H
authors.initials: CH
authors.lastname: Scharloo-Karels
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: M A A
authors.initials: MAA
authors.lastname: de Jong
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Oldenborg
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: M W
authors.initials: MW
authors.lastname: Kolff
authors.affiliation: Department of Radiation Oncology and Hyperthermia, Institute Verbeeten, Tilburg, The Netherlands.
authors.forename: S B
authors.initials: SB
authors.lastname: Oei
authors.affiliation: Department of Surgery, Antoni van Leeuwenhoek Hospital, Amsterdam, The Netherlands.
authors.forename: F
authors.initials: F
authors.lastname: van Coevorden
authors.affiliation: Department of Radiation Oncology, Hyperthermia Unit, Erasmus MC-Cancer Institute, Rotterdam, The Netherlands.
authors.forename: G C
authors.initials: GC
authors.lastname: van Rhoon
authors.affiliation: Department of Radiation Oncology, Hyperthermia Unit, Erasmus MC-Cancer Institute, Rotterdam, The Netherlands.
authors.forename: E A
authors.initials: EA
authors.lastname: Baartman
authors.affiliation: Dutch Cochrane Center, Utrecht, The Netherlands.
authors.forename: R J
authors.initials: RJ
authors.lastname: Scholten
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Crezee
authors.affiliation: Academic Medical Center, Department of Radiation Oncology and Hyperthermia, Amsterdam, The Netherlands. Electronic address: g.vantienhoven@amc.uva.nl.
authors.forename: G
authors.initials: G
authors.lastname: van Tienhoven
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001943:Breast Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000532:radiotherapy* / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D006394:Hemangiosarcoma / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008875:Middle Aged; D015999:Multivariate Analysis; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local; D009381:Neoplasms, Radiation-Induced / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D011878:Radiotherapy / Q000009:adverse effects; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D047368:Tumor Burden
keywords: Breast cancer; Local control; Radiation-induced angiosarcoma; Radiotherapy; Surgery