title: Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study.
abstract: OBJECTIVE
  A postsurgical "stage-based" protocol for ependymoma was designed.
  
  
  METHODS
  Children were given: (1) focal hyperfractionated radiotherapy (HFRT) if with no evidence of disease (NED), or (2) 4 courses with VEC followed by HFRT for residual disease (ED). HFRT dose was 70.4 Gy (1.1 Gy/fraction b.i.d.); VEC consisted of VCR 1.5 mg/m2 1/w, VP16 100 mg/m2/day x 3, CTX 3 g/m2 d 1. When feasible, second-look surgery was recommended.
  
  
  RESULTS
  Sixty-three consecutive children were enrolled: 46 NED, 17 ED; the tumor was infratentorial in 47 and supratentorial in 16, with spinal metastasis in 1. Of NED patients, 35 of 46 have been treated with HFRT; 8 received conventionally fractionated radiotherapy, and 3 received no treatment. Of the 17 ED patients, 9 received VEC + HFRT; violations due to postsurgical morbidity were as follows: HFRT only (2), conventionally fractionated radiotherapy (3) + VEC (2), and no therapy (1). Objective responses to VEC were seen in 54%; objective responses to RT were seen in 75%. Overall survival and progression-free survival at 5 years for all 63 children were 75% and 56%, respectively; for the NED subgroup, 82% and 65%; and for the ED subgroup, 61% and 35%, respectively. All histologies were centrally reviewed. At multivariate analysis, grading, age, and site proved significant for prognosis.
  
  
  CONCLUSIONS
  HFRT, despite the high total dose adopted, did not change the prognosis of childhood ependymoma as compared to historical series: New radiotherapeutic approaches are needed to improve local control. Future ependymoma strategies should consider grading when stratifying treatment indications.
authors.affiliation: Department of Pediatric Oncology, Istituto Nazionale Tumori, Milano, Italy. maura.massimino@istitutotumori.mi.it
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Felice
authors.initials: F
authors.lastname: Giangaspero
authors.forename: Alessandro
authors.initials: A
authors.lastname: Sandri
authors.forename: Pinuccia
authors.initials: P
authors.lastname: Valagussa
authors.forename: Giorgio
authors.initials: G
authors.lastname: Perilongo
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Garrè
authors.forename: Umberto
authors.initials: U
authors.lastname: Ricardi
authors.forename: Marco
authors.initials: M
authors.lastname: Forni
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Genitori
authors.forename: Giovanni
authors.initials: G
authors.lastname: Scarzello
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Salvina
authors.initials: S
authors.lastname: Barra
authors.forename: Maurizio
authors.initials: M
authors.lastname: Mascarin
authors.forename: Bianca
authors.initials: B
authors.lastname: Pollo
authors.forename: Martina
authors.initials: M
authors.lastname: Gardiman
authors.forename: Armando
authors.initials: A
authors.lastname: Cama
authors.forename: Pierina
authors.initials: P
authors.lastname: Navarria
authors.forename: Maurizio
authors.initials: M
authors.lastname: Brisigotti
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Rita
authors.initials: R
authors.lastname: Balter
authors.forename: Paola
authors.initials: P
authors.lastname: Fidani
authors.forename: Maurizio
authors.initials: M
authors.lastname: Stefanelli
authors.forename: Roberta
authors.initials: R
authors.lastname: Burnelli
authors.forename: Paolo
authors.initials: P
authors.lastname: Potepan
authors.forename: Marta
authors.initials: M
authors.lastname: Podda
authors.forename: Guido
authors.initials: G
authors.lastname: Sotti
authors.forename: Enrico
authors.initials: E
authors.lastname: Madon
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy* / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage; D019583:Dose Fractionation, Radiation; D004806:Ependymoma / Q000188:drug therapy* / Q000532:radiotherapy* / Q000601:surgery; D005047:Etoposide / Q000008:administration & dosage; D005240:Feasibility Studies; D006801:Humans; D015192:Infratentorial Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D010349:Patient Compliance; D011446:Prospective Studies; D018714:Radiotherapy, Adjuvant; D015173:Supratentorial Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D016019:Survival Analysis; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide