title: Evolving treatment strategies for parameningeal rhabdomyosarcoma: the experience of the Istituto Nazionale Tumori of Milan.
abstract: BACKGROUND
  Ninety pediatric patients with parameningeal rhabdomyosarcoma (PM-RMS) were treated between 1970 and 2002 at the Istituto Nazionale Tumori, Milan.
  
  
  METHODS
  Combined treatment was implemented in high-risk patients with whole-brain irradiation and intrathecal chemotherapy between 1978 and 1981 and intrathecal methotrexate alone between 1982 and 1987. Radiotherapy was given with conventional fractionation until 1988 and hyperfractionated accelerated radiotherapy (HART) thereafter.
  
  
  RESULTS
  The 5-year disease-free survival was 41.3%, increasing from 15.4% (1970-1977) to 55.9% (1989-2002) for localized cases. Local failure rates were 41% and 24% with conventional fractionation and HART, respectively. In patients treated with HART, acute toxicities were more pronounced, but the treatment compliance was better. Severe radiation-related late sequelae were observed.
  
  
  CONCLUSIONS
  The outcome for patients with PM-RMS has improved over the years but is still not as satisfactory as that for patients with RMS of other sites. Thanks to improvements in radiotherapy techniques and chemotherapy efficacy, meningeal prophylaxis is no longer necessary. Although more evidence is needed to confirm our data, the results obtained with twice-daily HART seem promising.
authors.affiliation: Pediatric Oncology Unit, Istituto Nazionale Tumori, Via G. Venezian, 1-20133 Milano MI, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Meazza
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.forename: Roberto
authors.initials: R
authors.lastname: Luksch
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Graziella
authors.initials: G
authors.lastname: Cefalo
authors.forename: Daniela
authors.initials: D
authors.lastname: Polastri
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Marta
authors.initials: M
authors.lastname: Podda
authors.forename: Giulio
authors.initials: G
authors.lastname: Cantù
authors.forename: Davide
authors.initials: D
authors.lastname: Scaramuzza
authors.forename: Franca
authors.initials: F
authors.lastname: Fossati-Bellani
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000517:prevention & control; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D016371:Cranial Irradiation; D019583:Dose Fractionation, Radiation; D005260:Female; D006801:Humans; D007223:Infant; D007558:Italy / Q000453:epidemiology; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D009364:Neoplasm Recurrence, Local; D009382:Neoplasms, Unknown Primary / Q000401:mortality / Q000628:therapy*; D018714:Radiotherapy, Adjuvant; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D016896:Treatment Outcome