title: Ewing's sarcoma family of tumors in Finland during 1990-2009: a population-based study.
abstract: BACKGROUND
  Ewing's sarcoma family of tumors (ESFTs) are rare bone and soft tissue tumors characterized by specific genetic alterations. Our aim was to carry out a nationwide analysis of ESFT, to survey the treatments used and to report the five-year disease specific and event-free survival rates (EFS and DSS).
  
  
  METHODS
  The study data was gathered from the Finnish National Cancer Registry and all five University Hospitals and consisted of 76 bone and soft tissue ESFT patients diagnosed during 1990-2009. Their medical records were reviewed and data on their disease, treatments, complications and outcome were analyzed.
  
  
  RESULTS
  The five-year EFS and DSS of patients with localized disease at diagnosis (n = 57) were 70% and 60%, respectively. Factors contributing to DSS and EFS were the axial vs. peripheral site of primary tumor and adequate surgical resection of the primary tumor. DSS was also affected by patient's age at diagnosis and the treatment employed. The five-year DSS of patients with metastatic disease at diagnosis (n = 19) was 33% and both preoperative and high dose chemotherapy were associated with improved survival.
  
  
  CONCLUSIONS
  Population-based studies including both bone and soft tissue ESFTs are few. In this nationwide, population-based study on Finnish bone and soft tissue ESFT patients, we find their treatment successful and results comparable to those previously published. Absence of metastases, young age at diagnosis and a peripheral primary tumor site were associated with a better prognosis. It seems that surgical resection of the primary tumor should be performed whenever adequate resection margins can be achieved. The role of high dose chemotherapy merits further studies in this setting.
authors.affiliation: Faculty of Medicine, University of Helsinki, Helsinki, Finland. joni.serlo@helsinki.fi
authors.forename: Joni A
authors.initials: JA
authors.lastname: Serlo
authors.forename: Ilkka J
authors.initials: IJ
authors.lastname: Helenius
authors.forename: Mika
authors.initials: M
authors.lastname: Sampo
authors.forename: Kim
authors.initials: K
authors.lastname: Vettenranta
authors.forename: Ulla M
authors.initials: UM
authors.lastname: Saarinen-Pihkala
authors.forename: Sanna-Maria
authors.initials: SM
authors.lastname: Kivivuori
authors.forename: Pekka
authors.initials: P
authors.lastname: Riikonen
authors.forename: Aarne
authors.initials: A
authors.lastname: Kivioja
authors.forename: Tom
authors.initials: T
authors.lastname: Böhling
authors.forename: Markku
authors.initials: M
authors.lastname: Kallajoki
authors.forename: Ari
authors.initials: A
authors.lastname: Ristimäki
authors.forename: Kaija
authors.initials: K
authors.lastname: Vasama
authors.forename: Maija
authors.initials: M
authors.lastname: Tarkkanen
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D005387:Finland / Q000453:epidemiology; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D012042:Registries / Q000706:statistics & numerical data; D012512:Sarcoma, Ewing / Q000453:epidemiology* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis; D055815:Young Adult