title: Surgically treated patients with axial and peripheral Ewing's sarcoma family of tumours: A population based study in Finland during 1990-2009.
abstract: BACKGROUND
  The surgical treatment of Ewing's sarcoma family tumours (ESFTs) is challenging especially with axial tumours. The aim of the study was to analyse surgical treatment and outcome in a nationwide, population-based material consisting of surgically treated axial and peripheral ESFTs of bone and soft tissue.
  
  
  METHODS
  The data were collected from the Finnish National Cancer Registry and the medical records of patients diagnosed during 1990-2009. Fifty-seven patients with surgically treated ESFTs were included, 22 with an axial and 35 with a peripheral primary tumours. The surgical treatment, its complications, survival and prognostic factors were analysed.
  
  
  RESULTS
  Fifty-four patients underwent surgery with a curative intent and three underwent de-bulking operations. Bone reconstruction was performed in six patients with an axial and 15 with a peripheral tumour. Positive resection margins were associated with a worse five-year local relapse-free survival (33% vs. 84% for those with resection margins free of tumour cells, p = 0.003). The five-year sarcoma-specific survival was affected only by an axial location of the primary (61% vs. 89% for those with a peripheral tumour, p = 0.031). The late complications were mainly associated with bone reconstruction and more frequent among patients with a peripheral compared to an axial tumour (p = 0.031).
  
  
  CONCLUSIONS
  In the treatment of ESFTs, achieving adequate resection margins is crucial to avoid local relapses. Surgical complications are common particularly with bone reconstruction.
authors.affiliation: Department of Oncology, Helsinki University Central Hospital, Haartmaninkatu 4, PL 180, HUS, 00029 Helsinki, Finland; Department of Pediatric Orthopedic Surgery, Turku University Hospital, Kiinamyllynkatu 4-8, PL 52, 20521 Turku, Finland. Electronic address: joni.serlo@helsinki.fi.
authors.forename: J
authors.initials: J
authors.lastname: Serlo
authors.affiliation: Department of Pediatric Orthopedic Surgery, Turku University Hospital, Kiinamyllynkatu 4-8, PL 52, 20521 Turku, Finland.
authors.forename: I
authors.initials: I
authors.lastname: Helenius
authors.affiliation: Department of Pediatrics, Helsinki University Central Hospital, Stenbäckinkatu 11, PL 281, HUS, 00029 Helsinki, Finland.
authors.forename: K
authors.initials: K
authors.lastname: Vettenranta
authors.affiliation: Department of Pediatrics, Kuopio University Hospital, Puijonlaaksontie 2, PL 100, KYS, 70029 Kuopio Finland.
authors.forename: M
authors.initials: M
authors.lastname: Perkkiö
authors.affiliation: Department of Pediatrics, Kuopio University Hospital, Puijonlaaksontie 2, PL 100, KYS, 70029 Kuopio Finland.
authors.forename: P
authors.initials: P
authors.lastname: Riikonen
authors.affiliation: Department of Oncology, Helsinki University Central Hospital, Haartmaninkatu 4, PL 180, HUS, 00029 Helsinki, Finland.
authors.forename: M
authors.initials: M
authors.lastname: Sampo
authors.affiliation: Department of Oncology, Helsinki University Central Hospital, Haartmaninkatu 4, PL 180, HUS, 00029 Helsinki, Finland.
authors.forename: M
authors.initials: M
authors.lastname: Tarkkanen
mesh_terms: D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000532:radiotherapy / Q000601:surgery*; D001842:Bone and Bones / Q000473:pathology* / Q000601:surgery*; D050281:Bones of Lower Extremity / Q000473:pathology / Q000601:surgery; D001133:Bones of Upper Extremity / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D019583:Dose Fractionation, Radiation; D005260:Female; D005387:Finland; D005500:Follow-Up Studies; D006801:Humans; D023821:Limb Salvage / Q000706:statistics & numerical data; D008297:Male; D008499:Medical Records; D016016:Proportional Hazards Models; D018714:Radiotherapy, Adjuvant; D012042:Registries; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000532:radiotherapy / Q000601:surgery*; D013131:Spine / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult
keywords: Axial; Ewing; Peripheral; Prognosis; Sarcoma