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Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience |
Bulletin du Cancer. Volume 95, Number 11, 1021-8, novembre 2008, Article original
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 Résumé
Texte intégral
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Author(s) : M Diepold, Annie Rey, Odile Oberlin, Nathalie Boulay, Christophe Bergeron, Christophe Glorion, Pierre Mary, Marie-José Terrier-Lacombe, Sylvie Helfre, Jean-Louis Habrand, Jean-Claude Gentet, Anne Sophie Defachelles, Caroline Thomas, Hervé Brisse, Gaëlle Pierron, Daniel Orbach |
Summary : Patients and methodsForty-six patients with localised RMS of the limbs entered the MMT 89 and 95 study in France. We studied potential risk factors that were predictive of relapse and survival to propose a therapeutic approach of surgery and radiotherapy appropriate to the risk of relapse.ResultsMedian age at diagnosis was 6.5 years [9 months to 15.5 years]. At time of diagnosis, 43% had marginal surgery and only 13% radical intervention. Primary re-excision was performed in 12% of the patients. All patients received chemotherapy, 43% had second look surgery and 37% received radiotherapy. Fifty-four percent of all tumors relapsed: local relapse 36%, nodes l8%, metastatic 40%, local and metastatic 16%. Estimated overall 5-year event-free survival (EFS) and overall survival (OS) were 40 and 57%, respectively.ConclusionsPrognosis of RMS of the limbs is bad but only 37% of the patients had radiotherapy. We could define patients with very high risk among those with limbs RMS as nodal involvement (5 years overall survival OS 22%), alveolar histology (OS 38%) and site of hand and foot (4 survivors out of 10 patients). In further studies, these patients should be treated even more aggressive with early surgery followed by re-excision if necessary, chemotherapy including alkylating agents and systematic radiotherapy. |
Keywords : rhabdomyosarcoma, limbs, children, adolescents, radiotherapy |
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