title: Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study.
abstract: OBJECTIVE
  The aim of the CWS 96 Study was to achieve an optimal treatment in children and adolescents with soft tissue sarcoma (STS) implementing a further refinement of risk-adapted allocation to chemotherapy, surgery and radiotherapy.
  
  
  METHODS
  Treatment stratification was based on tumour histology, TNM status, postsurgical stage, localisation and age. Local tumour control was ensured by surgery and risk-adapted radiotherapy.
  
  
  RESULTS
  From 1995 to 2002, 89 patients were registered in Austria. The 3-year event-free survival (EFS) and overall survival rates (OS) were 63% +/- 6% and 71% +/- 6%, respectively. 59/89 patients had localised RMS-like (rhabdomayosarcoma) STS (EFS 73% +/- 7%), 14 had localised NON-RMS STS (EFS 54% +/- 16%) and 15 patients had metastatic disease at diagnosis (EFS 33% +/- 12%), 1 patient had fibromatosis. The EFS rates at 3 years in patients with localised RMS-like tumours according to risk group were 92% +/- 8% for low and standard risk (12 patients) and 67% +/- 8% for high risk (47 patients). Favourable primary tumour sites of nonmetastatic RMS-like STS i.e. orbit, head/neck nonparameningeal or genitourinary non-bladder/prostate were diagnosed in 15 patients (1/15 patients died). In 44 patients with unfavourable localisation such as parameningeal, genitourinary bladder/prostate, extremity and others, 7 deceased. The 3 year EFS according to histology in patients with RMS-like STS was 61% +/- 11% for RME (embryonal RMS ) (28 patients) and 71% +/- 15% for RMA (alveolar RMS) (10 patients). The most common treatment failure was local relapse occurring in 21% of patients in the high-risk group.
  
  
  CONCLUSIONS
  Risk-adapted individualisation of treatment led to a reduction of chemotherapy in the low and standard risk group without compromising survival. The outcome of RME and RMA was similar in this cohort of patients. These preliminary results after a median observation time of 2.5 years confirm the CWS 96 strategy.
authors.affiliation: CCRI Children's Cancer Research Institute--St. Anna Kinderspital, Wien/Osterreichische Studienzentrale, Vienna, Austria.
authors.forename: Ditha
authors.initials: D
authors.lastname: Modritz
authors.forename: Ruth
authors.initials: R
authors.lastname: Ladenstein
authors.forename: Ulrike
authors.initials: U
authors.lastname: Pötschger
authors.forename: Gabriele
authors.initials: G
authors.lastname: Amman
authors.forename: Karin
authors.initials: K
authors.lastname: Dieckmann
authors.forename: Ernst
authors.initials: E
authors.lastname: Horcher
authors.forename: Christian
authors.initials: C
authors.lastname: Urban
authors.forename: Bernhard
authors.initials: B
authors.lastname: Meister
authors.forename: Klaus
authors.initials: K
authors.lastname: Schmitt
authors.forename: Regina
authors.initials: R
authors.lastname: Jones
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Kaulfersch
authors.forename: Hans
authors.initials: H
authors.lastname: Haas
authors.forename: Reinhard
authors.initials: R
authors.lastname: Moser
authors.forename: Olaf
authors.initials: O
authors.lastname: Stöllinger
authors.forename: Martina
authors.initials: M
authors.lastname: Peham
authors.forename: Helmut
authors.initials: H
authors.lastname: Gadner
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
authors.forename: Jörn
authors.initials: J
authors.lastname: Treuner
mesh_terms: D000293:Adolescent; D000328:Adult; D001317:Austria / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D012509:Sarcoma / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome