title: The use of paediatric chemotherapy protocols at full dose is both a rational and feasible treatment strategy in adults with Ewing's family tumours.
abstract: BACKGROUND
  Ewing's sarcoma and primitive neuroectodermal tumour (ES/PNET) are rare, limiting opportunities for therapy studies in adults. Chemotherapy regimens adapted from paediatric studies are often used for adults but concerns about poor outcome and treatment toxicity may adversely affect drug dose intensity. We present our experience using a paediatric protocol at full dose.
  
  
  METHODS
  Records of 34 patients with ES/PNET who received the IVAD chemotherapy regimens were reviewed. Received drug dose intensity, toxicity and survival data were collected.
  
  
  RESULTS
  Received dose intensity in 30 evaluable patients was 0.92 compared to the standard IVAD schedule. Myelosuppression was the major toxicity, 83% of patients experienced grade 4 neutropenia. There was no major renal or cardiac toxicity. In patients without metastases at presentation, five-year overall survival was 63% and progression free survival was 39%. Tumour burden at presentation was statistically significantly associated with survival (P = 0.002). The five-year survival rate of 80% in patients presenting with low volume non metastatic disease was equivalent to published paediatric series.
  
  
  CONCLUSIONS
  Although the IVAD chemotherapy regimens are myelotoxic in adults, they can be given safely. We recommend that adults with ES/PNET should be included in current multicentre, multidisciplinary treatment studies directed at children.
authors.affiliation: Sarcoma Unit, Royal Marsden NHS Trust, London, UK.
authors.forename: M W
authors.initials: MW
authors.lastname: Verrill
authors.forename: I R
authors.initials: IR
authors.lastname: Judson
authors.forename: E
authors.initials: E
authors.lastname: Wiltshaw
authors.forename: J M
authors.initials: JM
authors.lastname: Thomas
authors.forename: C L
authors.initials: CL
authors.lastname: Harmer
authors.forename: C
authors.initials: C
authors.lastname: Fisher
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy* / Q000401:mortality; D001932:Brain Neoplasms / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000008:administration & dosage; D018572:Disease-Free Survival; D004305:Dose-Response Relationship, Drug; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005047:Etoposide / Q000008:administration & dosage; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007262:Infusions, Intravenous; D007275:Injections, Intravenous; D008297:Male; D015080:Mesna / Q000008:administration & dosage; D008875:Middle Aged; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy*; D009503:Neutropenia / Q000139:chemically induced; D012512:Sarcoma, Ewing / Q000188:drug therapy* / Q000401:mortality; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D015080:Mesna; D007069:Ifosfamide