Paediatric and adolescent alveolar soft part sarcoma: A joint series from European cooperative groups

D Orbach, B Brennan, M Casanova, C Bergeron, V Mosseri, N Francotte, M Van Noesel, A Rey, G Bisogno, G Pierron, A Ferrari

Research output: Contribution to journalArticlepeer-review

53 Citations (Scopus)


BACKGROUND: Alveolar soft part sarcomas (ASPS) are generally chemo- and radio-resistant mesenchymal tumours, with no standardized treatment guidelines. We describe the clinical behaviour of paediatric ASPS and compare these features to previously reported adult series.

PATIENTS AND METHODS: The clinical data of 51 children and adolescents with ASPS, prospectively enrolled in or treated according to seven European Paediatric trials were analysed.

RESULTS: Median age was 13 years [range: 2-21]. Primary sites included mostly limbs (63%). IRS post-surgical staging was: IRS-I (complete resection) 35%, II (microscopic residual disease) 20%, III (gross residual disease) 18% and IV (metastases) 27%. Only 3 of the 18 evaluable patients (17%) obtained a response to conventional chemotherapy. After a median follow-up of 126 months (range: 9-240), 14/18 patients with IRS-I tumour, 10/10 IRS-II, 7/9 IRS-III and 2/14 IRS-IV were alive in remission. Sunitinib treatment achieved two very good partial responses in four patients. Ten-year overall survival (OS) and event free survival (EFS) was 78.0 ± 7% and 62.8 ± 7% respectively. Stage IV, size >5 cm and T2 tumours had a poorer outcome, but only IRS staging was an independent prognostic factor.

CONCLUSIONS: ASPS is a very rare tumour frequently arising in adolescents and in the extremities, and chemo resistant. Local surgical control is critical. ASPS is a poorly chemo sensitive tumour. For IRS-III/IV tumours, delayed radical local therapies including surgery are essential. Metastatic patients had a poor prognosis but targeted therapies showed promising results.

Original languageEnglish
Pages (from-to)1826-32
Number of pages7
JournalPediatric blood & cancer
Issue number11
Publication statusPublished - Jul 2013
Externally publishedYes


  • Adolescent
  • Antineoplastic Agents/therapeutic use
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Disease-Free Survival
  • Europe
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Proportional Hazards Models
  • Radiotherapy
  • Sarcoma, Alveolar Soft Part/mortality
  • Soft Tissue Neoplasms/mortality
  • Young Adult


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