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Modritz, D; Ladenstein, R; Pötschger, U; Amman, G; Dieckmann, K; Horcher, E; Urban, C; Meister, B; Schmitt, K; Jones, R; Kaulfersch, W; Haas, H; Moser, R; Stöllinger, O; Peham, M; Gadner, H; Koscielniak, E; Treuner, J.
Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study.
WIEN KLIN WOCHENSCHR. 2005; 117(5-6): 196-209.
Doi: 10.1007/s00508-004-5-0285-8
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- Co-Autor*innen der Med Uni Graz
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Urban Ernst-Christian
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- 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. CONCLUSION: 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.
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Adolescent -
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Adult -
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Austria - epidemiology
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Child - epidemiology
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Child, Preschool - epidemiology
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Cohort Studies - epidemiology
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Disease-Free Survival - epidemiology
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Female - epidemiology
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Humans - epidemiology
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Incidence - epidemiology
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Infant - epidemiology
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Infant, Newborn - epidemiology
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Male - epidemiology
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Prognosis - epidemiology
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Risk Assessment - methods
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Risk Factors - methods
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Sarcoma - diagnosis
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Survival Analysis - diagnosis
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Treatment Outcome - diagnosis
- Find related publications in this database (Keywords)
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soft tissue sarcoma
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adolescent
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Austria
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child
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combined modality therapy