[Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience]

Diepold, M; Rey, Annie; Oberlin, Odile; Boulay, Nathalie; Bergeron, Christophe; Glorion, Christophe; Mary, Pierre; Terrier-Lacombe, Marie-José; Helfre, Sylvie; Habrand, Jean-Louis; Gentet, Jean-Claude; Defachelles, Anne Sophie; Thomas, Caroline; Brisse, Hervé; Pierron, Gaëlle; Orbach, Daniel (2008). [Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience]. Bulletin du cancer, 95(11), pp. 1021-8. Paris: Association française pour l'étude du cancer 10.1684/bdc.2008.0739

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PATIENTS AND 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.

Item Type:	Journal Article (Further Contribution)
Division/Institute:	04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
UniBE Contributor:	Diepold, Miriam
ISSN:	0007-4551
ISBN:	19036673
Publisher:	Association française pour l'étude du cancer
Language:	English
Submitter:	Anette van Dorland
Date Deposited:	04 Oct 2013 15:03
Last Modified:	05 Dec 2022 14:19
Publisher DOI:	10.1684/bdc.2008.0739
PubMed ID:	19036673
Web of Science ID:	000261488100007
URI:	https://boris.unibe.ch/id/eprint/27185 (FactScience: 104901)