NON-METASTATIC UNRESECTED PAEDIATRIC NON-RHABDOMYOSARCOMA SOFT TISSUE SARCOMAS: RESULTS OF A POOLED ANALYSIS FROM UNITED STATES AND EUROPEAN GROUPS
- Ferrari, A
- Miceli, R
- Rey, A
- Oberlin, O
- Orbach, D
- Brennan, B
- Mariani, L
- Carli, MODESTO OTTAVIANO
- Bisogno, Gianni
- Cecchetto, Giovanni
- De Salvo, Gl
- Casanova, M
- Vannoesel, Mm
- Kelsey, A
- Stevens, Mc
- Devidas, M
- Pappo, As
- Spunt, S. L.
DOIAbstract
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...
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