title: Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience.
abstract: BACKGROUND
  Infantile fibrosarcoma (IFS) is a very rare disease occurring in young infants characterised by a high local aggressiveness but overall with a favourable survival. To try to reduce the total burden of therapy, the European pediatric Soft tissue sarcoma Study Group has developed conservative therapeutic recommendations according to initial resectability.
  
  
  METHODS
  Between 2005 and 2012, children with localised IFS were prospectively registered. Initial surgery was suggested only if possible without mutilation. Patients with initial complete (IRS-group I/R0) or microscopic incomplete (group II/R1) resection had no further therapy. Patients with initial inoperable tumour (group III/R2) received first-line vincristine-actinomycin-D chemotherapy (VA). Delayed conservative surgery was planned after tumour reduction. Aggressive local therapy (mutilating surgery or external radiotherapy) was discouraged.
  
  
  RESULTS
  A total of 50 infants (median age 1.4 months), were included in the study. ETV6-NTRK3 transcript was present in 87.2% of patients where investigation was performed. According to initial surgery, 11 patients were classified as group I, 8 as group II and 31 as group III. VA chemotherapy was first delivered to 25 children with IRS-III/R2 and one with IRS-II/R1 disease. Response rate to VA was 68.0%. Mutilating surgery was only performed in three cases. After a median follow-up of 4.7 years (range 1.9-9.0), 3-year event-free survival and overall survival were respectively 84.0% (95% confidence interval [CI] 70.5-91.7) and 94.0% (95% CI 82.5-98.0).
  
  
  CONCLUSIONS
  Conservative therapy is possible in IFS as only three children required mutilating surgery, and alkylating or anthracycline based chemotherapy was avoided in 71.0% of patients needing chemotherapy. VA regimen should be first line therapy in order to reduce long term effects.
authors: Department of Pediatric, Adolescent and Young Adult Oncology, Institut Curie, Paris, France. Electronic address: daniel.orbach@curie.fr.
authors: Daniel
authors: D
authors: Orbach
authors: Department of Pediatric Oncology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors: Bernadette
authors: B
authors: Brennan
authors: Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy.
authors: Angela
authors: A
authors: De Paoli
authors: Paediatric Oncology, Hospital Universitario Vall d'Hebron, Barcelona, Spain.
authors: Soledad
authors: S
authors: Gallego
authors: Department of Pediatric Oncology, University Children's Hospital, Brno, Czech Republic.
authors: Peter
authors: P
authors: Mudry
authors: Department of Pediatrics, CHC-Clinique Esperance, Montegnée, Belgium.
authors: Nadine
authors: N
authors: Francotte
authors: Princess Máxima Center for Pediatric Oncology, Utrecht, Netherlands.
authors: Max
authors: M
authors: van Noesel
authors: Department of Diagnostic Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
authors: Anna
authors: A
authors: Kelsey
authors: Pathology Department, Padova University, Padova, Italy.
authors: Rita
authors: R
authors: Alaggio
authors: Pathology Department, Institut d'Hematologie et d'Oncologie Pediatrique, Centre Léon Bérard, Lyon, France.
authors: Dominique
authors: D
authors: Ranchère
authors: Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy.
authors: Gian Luca
authors: GL
authors: De Salvo
authors: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors: Michela
authors: M
authors: Casanova
authors: Department of Pediatric Oncology, Institut d'Hematologie et d'Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France.
authors: Christophe
authors: C
authors: Bergeron
authors: Department of Pediatric Oncology, Emma Children's Hospital-Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands.
authors: Johannes H M
authors: JH
authors: Merks
authors: Department of Pediatric Oncology, Children's Hospital for Wales, Heath Park, Cardiff, United Kingdom.
authors: Meriel
authors: M
authors: Jenney
authors: Department of Pediatric Oncology, Royal Hospital for Children, University of Bristol, United Kingdom.
authors: Michael C G
authors: MC
authors: Stevens
authors: Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.
authors: Gianni
authors: G
authors: Bisogno
authors: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors: Andrea
authors: A
authors: Ferrari
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant / Q000379:methods; D003131:Combined Modality Therapy / Q000379:methods; D017281:Cost of Illness; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D005240:Feasibility Studies; D005260:Female; D005354:Fibrosarcoma / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008207:Lymphatic Metastasis; D008297:Male; D009367:Neoplasm Staging; D011446:Prospective Studies; D012086:Reoperation; D012983:Soft Tissue Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D057832:Watchful Waiting
chemical_list: D000970:Antineoplastic Agents; D003609:Dactinomycin; D014750:Vincristine
keywords: Cancer; Chemotherapy; ETV6-NTRK3 transcript; Infant; Infantile fibrosarcoma; Newborn