title: Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups.
abstract: BACKGROUND
  Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with initially unresected tumours represent a particular subset of patients with a poor outcome. Various international research groups pooled their data in a joint study in order to investigate prognostic variables and treatment modalities.
  
  
  METHODS
  The study population consisted of 304 patients <21 years old treated between 1980 and 2005 using a multimodality therapeutic strategy.
  
  
  RESULTS
  Synovial sarcoma and malignant peripheral nerve sheath tumour (MPNST) were the most frequent histotypes. Most patients received initial chemotherapy: major responses were recorded in 41% and minor in 16% of cases. Overall survival (OS) was 60.0% and 51.5% at 5 and 10 years, respectively, and it was significantly associated with patient's age, histological subtype, tumour site and size, quality of delayed surgical resection, radiotherapy administration and response to induction chemotherapy. MPNST associated to neurofibromatosis type 1 was the tumour type with the worst rate of response to chemotherapy and the worst outcome.
  
  
  CONCLUSIONS
  In unresected NRSTS patients, radiotherapy and delayed surgery are of crucial importance. Patients who respond to chemotherapy have better chance of survival. However, given the relatively poor prognosis, research on intensive multimodal treatment approaches and novel strategies is warranted.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. andrea.ferrari@istitutotumori.mi.it
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Rosalba
authors.initials: R
authors.lastname: Miceli
authors.forename: Annie
authors.initials: A
authors.lastname: Rey
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.forename: Bernadette
authors.initials: B
authors.lastname: Brennan
authors.forename: Luigi
authors.initials: L
authors.lastname: Mariani
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Gian Luca
authors.initials: GL
authors.lastname: De Salvo
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Max M
authors.initials: MM
authors.lastname: Vannoesel
authors.forename: Anna
authors.initials: A
authors.lastname: Kelsey
authors.forename: Michael C
authors.initials: MC
authors.lastname: Stevens
authors.forename: Meenakshi
authors.initials: M
authors.lastname: Devidas
authors.forename: Alberto S
authors.initials: AS
authors.lastname: Pappo
authors.forename: Sheri L
authors.initials: SL
authors.lastname: Spunt
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000209:etiology; D018317:Nerve Sheath Neoplasms / Q000473:pathology / Q000628:therapy; D012509:Sarcoma / Q000473:pathology / Q000628:therapy*; D013584:Sarcoma, Synovial / Q000628:therapy; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult