title: Improved survival for children with parameningeal rhabdomyosarcoma: results from the AIEOP soft tissue sarcoma committee.
abstract: BACKGROUND
  Parameningeal rhabdomyosarcoma (PM-RMS) is a rare, highly malignant pediatric tumor arising from locations adjacent to the meninges, from where it can spread intracranially.
  
  
  METHODS
  We reviewed 109 children with non-metastatic PM-RMS enrolled in the Italian RMS79, RMS88 and RMS96 protocols over a 24-year period. All patients received intensive chemotherapy and standard or hyperfractionated and accelerated radiotherapy. Some had delayed surgery.
  
  
  RESULTS
  Five-year overall survival rose from 40% in the RMS79 to 72% in the RMS88 and RMS96 protocols (P = 0.01), where more intensive chemotherapy and hyperfractionated accelerated radiotherapy (HART) was used. Delayed surgery after initial treatment was statistically associated with a better prognosis. Unfavorable tumor characteristics for RMS arising in other sites, for example, histology, invasiveness or node involvement, did not predict outcome for PM-RMS.
  
  
  CONCLUSIONS
  Outcome in PM-RMS patients enrolled in three consecutive Italian protocols has progressively improved, as a result of intensive chemotherapy, delayed surgery and, possibly, HART, though improved imaging and radiotherapeutic tools may have had a role as well.
authors.affiliation: Division of Hematology/Oncology, Department of Pediatrics, University Hospital of Padova, Padova, Italy. gianni.bisogno@unipd.it
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Costanza
authors.initials: C
authors.lastname: De Rossi
authors.forename: Yéssika
authors.initials: Y
authors.lastname: Gamboa
authors.forename: Guido
authors.initials: G
authors.lastname: Sotti
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Sandro
authors.initials: S
authors.lastname: Dallorso
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Milena
authors.initials: M
authors.lastname: Calderone
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008577:Meningeal Neoplasms / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D012208:Rhabdomyosarcoma / Q000401:mortality* / Q000628:therapy; D015996:Survival Rate