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Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience Volume 95, issue 11, novembre 2008

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Authors
Hôpital universitaire de Berne, Suisse, Département de biostatistiques, institut Gustave-Roussy, 94805 Villejuif, France, Département de pédiatrie, institut Gustave-Roussy, 94805 Villejuif, France, Département de pédiatrie, centre Léon-Bérard, 69373 Lyon, France, Service de chirurgie orthopédique, hôpital Necker, Assistance publique, 75015 Paris, France, Service de chirurgie orthopédique, hôpital Trousseau, Assistance publique, 75571 Paris, France, Département de pathologie, institut Gustave-Roussy, 94805 Villejuif, France, Département de radiothérapie, institut Curie, 75248 Paris, France, Département de pédiatrie, hôpital La Timone, 13385 Marseille, France, Département de pédiatrie, centre Oscar-Lambret, 59020 Lille, France, Service de pédiatrie, hôpital Hôtel-Dieu, 44093 Nantes, France, Département de radiologie, institut Curie, 75248 Paris, France, Laboratoire de génétique somatique, institut Curie, 75248 Paris, France, Département de pédiatrie, institut Curie, 75248 Paris, France

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.