Département de pédiatrie, Centre Léon-Bérard, 28, rue Laennec, 69373 Lyon Cedex 08.
Rhabdomyosarcoma is a rare tumour corresponding to 60-70% of soft tissue sarcomas in children. Significant advances in treatment have been made possible, and will be further obtained, by multicentric treatment protocols conducted in paediatric oncology centres. Overall survival and disease-free survival have been significantly improved over the past 30 years. In the meantime, diagnosis improvements have made the classification of rhabdomyosarcomas more complex. A review of European and American studies has evidenced a number of criteria that should be taken into account for selecting treatment strategy: histological examination (refined with the use of molecular biology) had proved very informative, suggesting a worse prognosis for alveolar rhabdomyosarcomas. Other criteria of interest are the tumour site (favourable or unfavourable), patient age (under or above 10), tumour size (± 5 cm), and disease stage. The number of sub-groups of patients requiring different, more adapted treatment strategies increases with the number of prognostic parameters to be considered. For convenient clinical management, patients will therefore be classified into 4 risk groups for systemic therapy (low, standard, high and very high risk), whereas local treatment strategies will take into account the whole set of prognostic criteria defined above.