title: Infant ependymoma in a 10-year AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) experience with omitted or deferred radiotherapy.
abstract: OBJECTIVE
  The protocols of the 1990s omitted or delayed irradiation, using upfront chemotherapy to spare the youngest children with ependymoma the sequelae of radiotherapy (RT). We treated 41 children under the age of 3 years with intracranial ependymoma between 1994 and 2003.
  
  
  METHODS
  After surgery, chemotherapy was given as follows: regimen I with four blocks of vincristine, high-dose methotrexate 5 g/m(2), and cyclophosphamide 1.5 g/m(2) alternating with cisplatin 90 mg/m(2) plus VP16 450 mg/m(2) for 14 months; subsequently, regimen II was used: VEC (VCR, VP16 300 mg/m(2), and cyclophosphamide 3 g/m(2)) for 6 months. Radiotherapy was planned for residual tumor after the completion of chemotherapy or for progression.
  
  
  RESULTS
  We treated 23 boys and 18 girls who were a median 22 months old; 14 were given regimen I, 27 were given regimen II; 22 underwent complete resection, 19 had residual tumor. Ependymoma was Grade 2 in 25 patients and Grade 3 in 16; tumors were infratentorial in 37 patients and supratentorial in 4. One child had intracranial metastases; 29 had progressed locally after a median 9 months. Event-free survival was 26% at 3 and 5 years and 23% at 8 years. One child died of sepsis, and another developed a glioblastoma 72 months after RT. Progression-free survival was 27% at 3, 5, and 8 years, and overall survival was 48%, 37%, and 28% at 3, 5, and 8 years, respectively. Of the 13 survivors, 6 never received RT; their intellectual outcome did not differ significantly in those children than in those without RT.
  
  
  CONCLUSIONS
  Our results confirm poor rates of event-free survival and overall survival for up-front chemotherapy in infant ependymoma. No better neurocognitive outcome was demonstrated in the few survivors who never received RT.
authors.affiliation: Department of Pediatrics, Fondazione IRCCS, 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: Salvina
authors.initials: S
authors.lastname: Barra
authors.forename: Felice
authors.initials: F
authors.lastname: Giangaspero
authors.forename: Cecilia
authors.initials: C
authors.lastname: Casali
authors.forename: Paolo
authors.initials: P
authors.lastname: Potepan
authors.forename: Concezio
authors.initials: C
authors.lastname: Di Rocco
authors.forename: Paolo
authors.initials: P
authors.lastname: Nozza
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Viscardi
authors.forename: Daniele
authors.initials: D
authors.lastname: Bertin
authors.forename: Veronica
authors.initials: V
authors.lastname: Biassoni
authors.forename: Armando
authors.initials: A
authors.lastname: Cama
authors.forename: Claudia
authors.initials: C
authors.lastname: Milanaccio
authors.forename: Piergiorgio
authors.initials: P
authors.lastname: Modena
authors.forename: Rita
authors.initials: R
authors.lastname: Balter
authors.forename: Giampiero
authors.initials: G
authors.lastname: Tamburrini
authors.forename: Paola
authors.initials: P
authors.lastname: Peretta
authors.forename: Maurizio
authors.initials: M
authors.lastname: Mascarin
authors.forename: Giovanni
authors.initials: G
authors.lastname: Scarzello
authors.forename: Paola
authors.initials: P
authors.lastname: Fidani
authors.forename: Giuseppe Maria
authors.initials: GM
authors.lastname: Milano
authors.forename: Iacopo
authors.initials: I
authors.lastname: Sardi
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Genitori
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Garrè
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002675:Child, Preschool; D003131:Combined Modality Therapy / Q000379:methods; D003520:Cyclophosphamide / Q000008:administration & dosage; D018572:Disease-Free Survival; D004806:Ependymoma / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007223:Infant; D015192:Infratentorial Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D007558:Italy; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D018365:Neoplasm, Residual; D015173:Supratentorial Neoplasms / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D008727:Methotrexate