title: Infantile fibrosarcoma: management based on the European experience.
abstract: OBJECTIVE
  To retrospectively analyze the clinical features and results of treatment in 56 infants with fibrosarcoma enrolled onto cooperative European protocols between 1979 and 2005 and treated with a combination of surgery and chemotherapy.
  
  
  METHODS
  We performed a retrospective case review of infants under the age of 2 years with fibrosarcoma treated between 1979 and 2005 in six European studies. Patients were staged according to the Intergroup Rhabdomyosarcoma Staging System international classification as a function of the type of initial surgery and the extent of disease and were treated with surgery and chemotherapy. Survival was calculated using the Kaplan-Meier method.
  
  
  RESULTS
  Primary tumor site was the limbs in 66% of patients; median tumor diameter was more than 5 cm in 63% of patients; and postoperative staging was as follows: group I, 22%; group II, 27%; group III, 47%; and group IV, 4%. Response rate to chemotherapy was 75%, and the specific response rate to vincristine-dactinomycin was 71%. Local control was obtained in 84% of patients. At the end of follow-up, 45% of survivors had been treated by surgery alone, 6% by chemotherapy alone, 46% by surgery and chemotherapy, and 2% by surgery, chemotherapy, and radiotherapy. The 5-year overall survival (OS) rate was 89%. The 5-year OS and event-free survival rates for localized patients were 89% and 81%, respectively.
  
  
  CONCLUSIONS
  Although complete resection is rarely feasible at diagnosis, conservative surgery remains the mainstay treatment for infantile fibrosarcoma. An alkylating agent-free and anthracycline-free regimen is usually effective and should be chosen as first-line chemotherapy for inoperable tumors. Overall prognosis is good, but progression or relapse, mainly local, remains possible.
authors.affiliation: Département de Pédiatrie, Institut Curie, 26 rue d'Ulm, 75005 Paris, France. daniel.orbach@curie.net
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.forename: Annie
authors.initials: A
authors.lastname: Rey
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Estelle
authors.initials: E
authors.lastname: Thebaud
authors.forename: Marcelo
authors.initials: M
authors.lastname: Scopinaro
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
mesh_terms: D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D005060:Europe; D005121:Extremities; D005260:Female; D005354:Fibrosarcoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012008:Recurrence; D016019:Survival Analysis