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Soft‐tissue sarcomas in children and adolescents with neurofibromatosis type 1

Ferrari, Andrea ; Bisogno, Gianni ; Macaluso, Alessandra ; Casanova, Michela ; D' Angelo, Paolo ; Pierani, Paolo ; Zanetti, Ilaria ; Alaggio, Rita ; Cecchetto, Giovanni ; Carli, Modesto

Cancer, April 2007, Vol.109(7), pp.1406-1412 [Rivista Peer Reviewed]

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  • Titolo:
    Soft‐tissue sarcomas in children and adolescents with neurofibromatosis type 1
  • Autore: Ferrari, Andrea ; Bisogno, Gianni ; Macaluso, Alessandra ; Casanova, Michela ; D' Angelo, Paolo ; Pierani, Paolo ; Zanetti, Ilaria ; Alaggio, Rita ; Cecchetto, Giovanni ; Carli, Modesto
  • Note di contenuto: BACKGROUND: Patients affected by neurofibromatosis type 1 (NF1) are at higher risk of developing soft-tissue sarcomas (STS) than the general population. The clinical findings and outcome in 43 children and adolescents with NF1 treated for STS in the Italian protocols between 1988 and 2004 are reported.METHODS: The study included 37 patients with neurogenic sarcomas (36 malignant peripheral nerve sheath tumors [MPNST], 1 triton tumor) and 6 cases of rhabdomyosarcoma (RMS). The prevalence of NF1 observed during the study period was 43% in the MPNST population and 1% in the RMS group.RESULTS: Most patients with neurogenic sarcomas had large, invasive tumors. Five-year event-free and overall survival rates were 19% and 28%, respectively. Two of 16 patients with evaluable disease responded to chemotherapy. All 6 RMS patients were
  • Fa parte di: Cancer, April 2007, Vol.109(7), pp.1406-1412
  • Soggetti: Rhabdomyosarcoma ; Malignant Peripheral Nerve Sheath Tumor ; Pediatric Soft Tissue Sarcomas ; Neurofibromatosis
  • Lingua: Inglese
  • Tipo: Articolo
  • Identificativo: ISSN: 0008-543X ; E-ISSN: 1097-0142 ; DOI: 10.1002/cncr.22533

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