title: [Hemangiosarcoma after breast-conserving therapy of breast cancer: report of four cases with molecular genetic diagnosis and literature review].
abstract: BACKGROUND
  Hemangiosarcomas of the breast represent a rare disease of the breast mainly occurring as secondary neoplasias with a latency of 5-10 years after primary treatment of breast cancer and are associated with an unfavourable prognosis. Radiation therapy, which is integrated within the concept of breast conserving therapy ranks as the main risk factor.
  
  
  METHODS
  In this report we describe the clinical course of 4 patients including their molecular genetic pattern and give a summary of the actual literature.
  
  
  RESULTS
  Hemangiosarcomas occur as a secondary neoplasm with a latency of 5-10 years after primary treatment of breast cancer and have an unfavorable prognosis. A genetic predisposition is assumed, but we could not find a significant role of tumor suppressor genes BRCA1, BRCA2 or p53 in our patients.
  
  
  CONCLUSIONS
  Due to limited data available for these tumors, recommendations for therapy include radical tumor resection achieving wide free margins and inconsistent regimens of chemo- and/or immunetherapy modalities. In the majority these are based on systemic therapy regimens for other cutaneous sarcomas, such as Kaposi's sarcoma. Efforts should be taken for a nation-wide systematic registration of all cases of post-irradiation hemangiosarcomas.
authors.affiliation: Frauenklinik, Universitätsklinikum Düsseldorf, Germany.
authors.forename: Carolin
authors.initials: C
authors.lastname: Nestle-Krämling
authors.forename: Edwin
authors.initials: E
authors.lastname: Bölke
authors.forename: Wilfried
authors.initials: W
authors.lastname: Budach
authors.forename: Matthias
authors.initials: M
authors.lastname: Peiper
authors.forename: Dieter
authors.initials: D
authors.lastname: Niederacher
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Janni
authors.forename: Claus Ferdinand
authors.initials: CF
authors.lastname: Eisenberger
authors.forename: Wolfram Trudo
authors.initials: WT
authors.lastname: Knoefel
authors.forename: Axel
authors.initials: A
authors.lastname: Scherer
authors.forename: Stephan Ernst
authors.initials: SE
authors.lastname: Baldus
authors.forename: Guido
authors.initials: G
authors.lastname: Lammering
authors.forename: Peter Arne
authors.initials: PA
authors.lastname: Gerber
authors.forename: Christiane
authors.initials: C
authors.lastname: Matuschek
mesh_terms: D000328:Adult; D000368:Aged; D024682:BRCA2 Protein / Q000235:genetics; D001943:Breast Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000532:radiotherapy* / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D004252:DNA Mutational Analysis; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D016153:Genes, Suppressor; D006394:Hemangiosarcoma / Q000175:diagnosis* / Q000235:genetics*; D006801:Humans; D015412:Mastectomy, Segmental; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000235:genetics; D009381:Neoplasms, Radiation-Induced / Q000175:diagnosis* / Q000235:genetics*; D016609:Neoplasms, Second Primary / Q000175:diagnosis* / Q000235:genetics*; D057089:Pathology, Molecular; D018714:Radiotherapy, Adjuvant / Q000009:adverse effects; D013524:Surgical Flaps; D016159:Tumor Suppressor Protein p53 / Q000235:genetics; D044767:Ubiquitin-Protein Ligases / Q000235:genetics
chemical_list: D024682:BRCA2 Protein; C551750:BRCA2 protein, human; D016159:Tumor Suppressor Protein p53; C111164:BRAP protein, human; D044767:Ubiquitin-Protein Ligases