Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study.
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.
Massimino
Maura
M
Department of Pediatric Oncology, Istituto Nazionale Tumori, Milano, Italy. maura.massimino@istitutotumori.mi.it
Gandola
Lorenza
L
Giangaspero
Felice
F
Sandri
Alessandro
A
Valagussa
Pinuccia
P
Perilongo
Giorgio
G
Garrè
Maria Luisa
ML
Ricardi
Umberto
U
Forni
Marco
M
Genitori
Lorenzo
L
Scarzello
Giovanni
G
Spreafico
Filippo
F
Barra
Salvina
S
Mascarin
Maurizio
M
Pollo
Bianca
B
Gardiman
Martina
M
Cama
Armando
A
Navarria
Pierina
P
Brisigotti
Maurizio
M
Collini
Paola
P
Balter
Rita
R
Fidani
Paola
P
Stefanelli
Maurizio
M
Burnelli
Roberta
R
Potepan
Paolo
P
Podda
Marta
M
Sotti
Guido
G
Madon
Enrico
E
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
D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide