[Localised rhabdomyosarcoma of the extremities in children and adolescents: results of the French experience].
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.
Diepold
M
M
Hôpital universitaire de Berne, Suisse.
Rey
Annie
A
Oberlin
Odile
O
Boulay
Nathalie
N
Bergeron
Christophe
C
Glorion
Christophe
C
Mary
Pierre
P
Terrier-Lacombe
Marie-José
MJ
Helfre
Sylvie
S
Habrand
Jean-Louis
JL
Gentet
Jean-Claude
JC
Defachelles
Anne Sophie
AS
Thomas
Caroline
C
Brisse
Hervé
H
Pierron
Gaëlle
G
Orbach
Daniel
D
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