title: [Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience].
abstract: METHODS
  Forty-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.
  
  
  RESULTS
  Median 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.
  
  
  CONCLUSIONS
  Prognosis 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.
authors.affiliation: Hôpital universitaire de Berne, Suisse.
authors.forename: M
authors.initials: M
authors.lastname: Diepold
authors.forename: Annie
authors.initials: A
authors.lastname: Rey
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.forename: Nathalie
authors.initials: N
authors.lastname: Boulay
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.forename: Christophe
authors.initials: C
authors.lastname: Glorion
authors.forename: Pierre
authors.initials: P
authors.lastname: Mary
authors.forename: Marie-José
authors.initials: MJ
authors.lastname: Terrier-Lacombe
authors.forename: Sylvie
authors.initials: S
authors.lastname: Helfre
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Habrand
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Gentet
authors.forename: Anne Sophie
authors.initials: AS
authors.lastname: Defachelles
authors.forename: Caroline
authors.initials: C
authors.lastname: Thomas
authors.forename: Hervé
authors.initials: H
authors.lastname: Brisse
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Pierron
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D005121:Extremities; D005602:France; D006801:Humans; D007223:Infant; D009364:Neoplasm Recurrence, Local; D011446:Prospective Studies; D012208:Rhabdomyosarcoma / Q000628:therapy*; D024162:Second-Look Surgery; D016896:Treatment Outcome