title: Salvage rates and prognostic factors after relapse in children and adolescents with initially localised synovial sarcoma.
abstract: BACKGROUND
  Previous studies have reported a poor outcome for synovial sarcoma patients whose tumours relapse.
  
  
  METHODS
  This study analysed 44 relapsing cases in a series of 118 consecutive patients <21 yr of age with non-metastatic synovial sarcoma prospectively enrolled in Italian paediatric protocols between 1979 and 2006. In an effort to identify a possible risk-adapted stratification enabling a better planning of second-line treatment, the relapsing patients' outcome was analysed vis-à-vis their clinical picture at onset, first-line treatments, clinical findings at the time of first relapse and second-line treatment modalities.
  
  
  RESULTS
  The first event was a local recurrence in only 15 cases, and metastatic in 29 (associated with local relapse too in 7 cases). The time to relapse ranged from 4 to 108 months (median 20 months). Overall survival was 29.7% and 21.0% five and ten years after relapsing, respectively. The variables influencing survival were the timing and type of relapse (combined) and the chances of a secondary remission, which correlated strongly with the feasibility of complete surgery.
  
  
  CONCLUSIONS
  Our study confirmed a largely unsatisfactory prognosis after recurrences in children and adolescents with synovial sarcoma: the chances of survival can be estimated on the basis of several variables for the purposes of planning risk-adapted salvage protocols. An aggressive surgical approach should be recommended. New effective systemic agents are warranted, and experimental therapies can be offered to patients with little chance of salvage.
authors: Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G. Venezian, Milan, Italy. andrea.ferrari@istitutotumori.mi.it
authors: Andrea
authors: A
authors: Ferrari
authors: Gian Luca
authors: GL
authors: De Salvo
authors: Patrizia
authors: P
authors: Dall'Igna
authors: Cristina
authors: C
authors: Meazza
authors: Francesco
authors: F
authors: De Leonardis
authors: Carla
authors: C
authors: Manzitti
authors: Maria Antonietta
authors: MA
authors: De Ioris
authors: Michela
authors: M
authors: Casanova
authors: Modesto
authors: M
authors: Carli
authors: Gianni
authors: G
authors: Bisogno
mesh_terms: D000293:Adolescent; D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016190:Carboplatin / Q000008:administration & dosage; D017024:Chemotherapy, Adjuvant; D002648:Child; D002986:Clinical Trials as Topic / Q000706:statistics & numerical data; D003131:Combined Modality Therapy; D003606:Dacarbazine / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007558:Italy / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D008297:Male; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D009364:Neoplasm Recurrence, Local / Q000401:mortality; D011379:Prognosis; D011446:Prospective Studies; D018714:Radiotherapy, Adjuvant; D012074:Remission Induction; D016879:Salvage Therapy / Q000706:statistics & numerical data; D013584:Sarcoma, Synovial / Q000401:mortality* / Q000556:secondary / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D005047:Etoposide; D003606:Dacarbazine; D004317:Doxorubicin; D016190:Carboplatin; D007069:Ifosfamide