title: Clinical management of secondary angiosarcoma after breast conservation therapy.
abstract: OBJECTIVE
  The aim of this paper is to summarize the treatment outputs of secondary angiosarcoma after breast conservation therapy at St. Eizabeth Cancer Centre, Slovakia.
  
  
  BACKGROUND
  Angiosarcoma of the breast is a rare but very aggressive malignant tumor of the vascular endothelium, characterized by rapidly proliferating and extensively infiltrating growth. Breast angiosarcoma may occur de novo, or as a complication of radiation therapy, or chronic lymphedema secondary to axillary lymph node dissection for mammary carcinoma. Radiotherapy in the treatment of breast cancer is associated with an increased risk of subsequent sarcoma.
  
  
  METHODS
  Retrospective study of medical records from the cancer databases was done in order to analyze the secondary breast angiosarcoma. This disease is an iatrogenic condition that warrants close follow-up and judicial use of radiotherapy in breast conserving therapy. Therefore, it is more prevalent in cases treated with radiotherapy, occurring especially in or adjacent to the radiation field. Clinical histories and follow-up data of identified patients after breast conservation therapy of invasive breast cancer were reviewed. In addition, a comprehensive literature review on diagnosis and treatment procedures was done in order to summarize state-of-the-art clinical approach.
  
  
  CONCLUSIONS
  Three cases of secondary angiosarcoma after breast conservation therapy (BCT) were identified among 4600 patients treated at St. Elizabeth Cancer Institute during previous 16 years (1995-2011). Secondary breast angiosarcoma was diagnosed in a median period of 11 years following primary radiotherapy, median age at the time of diagnosis was 75 years. Surgical treatment consisted of radical mastectomy. The first patient, a 56-year-old woman received neoadjuvant chemotherapy (docetaxel + gemcitabin), second one (75 year) was treated by radiotherapy (TD 26 Gy, 2 Gy per fraction), since chemotherapy was not indicated. The last patient (80 year) got adjuvant chemotherapy (paclitaxel). Average follow up of the patients was 31 months. As of 31 July 2012, our patients were doing well without evidence of recurrent disease after treatment.
  
  
  CONCLUSIONS
  Angiosarcoma remains a difficult management problem with poor loco-regional and distal control. In our study, an overall incidence rate of secondary breast angiosarcoma is 0.065%. Although the prognosis for this disease is poor (typical survival period is 14.5-34 months with a 5-year survival rate of approximately 15%), all the three patients treated at our institute are alive and disease-free at the end of reported period. Finally, it is assumed that the use of breast conserving therapy will increase the incidence of post-irradiation angiosarcoma but the small difference in risk of subsequent sarcoma of the breast cancer patients receiving radiotherapy does not suppress its benefit.
authors.affiliation: 1st Department of Oncology, Faculty of Medicine, Comenius University and St. Elisabeth Cancer Institute, Bratislava, Slovakia ; Department of Radiation Oncology, St. Elizabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Martina
authors.initials: M
authors.lastname: Zemanova
authors.affiliation: Department of Pathology, St. Elizabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Katarina
authors.initials: K
authors.lastname: Machalekova
authors.affiliation: Department of Radiation Oncology, St. Elizabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Monika
authors.initials: M
authors.lastname: Sandorova
authors.affiliation: Department of Radiation Oncology, St. Elizabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Elena
authors.initials: E
authors.lastname: Boljesikova
authors.affiliation: Department of Clinical Oncology, St. Elizabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Marta
authors.initials: M
authors.lastname: Skultetyova
authors.affiliation: 1st Department of Oncology, Faculty of Medicine, Comenius University and St. Elisabeth Cancer Institute, Bratislava, Slovakia.
authors.forename: Juraj
authors.initials: J
authors.lastname: Svec
authors.affiliation: Department of Nuclear Sciences and Applications, International Atomic Energy Agency, Vienna, Austria.
authors.forename: Andrej
authors.initials: A
authors.lastname: Zeman
keywords: Breast cancer; External-beam radiotherapy; Multimodality therapy; Radiationinduced late effects; Sarcoma