Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups

Andrea Ferrari, Rosalba Miceli, Annie Rey, Odile Oberlin, Daniel Orbach, Bernadette Brennan, Luigi Mariani, Modesto Carli, Gianni Bisogno, Giovanni Cecchetto, Gian Luca De Salvo, Michela Casanova, Max M Van Noesel, Anna Kelsey, Michael C Stevens, Meenakshi Devidas, Alberto S Pappo, Sheri L Spunt

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)724-31
Number of pages8
JournalEuropean Journal of Cancer
Issue number5
Publication statusPublished - Dec 2010
Externally publishedYes


  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols/therapeutic use
  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Neoplasm Recurrence, Local/etiology
  • Nerve Sheath Neoplasms/pathology
  • Sarcoma/pathology
  • Sarcoma, Synovial/therapy
  • Treatment Outcome
  • United States
  • Young Adult


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