Diffuse pontine gliomas in children: changing strategies, changing results? A mono-institutional 20-year experience.
Patients with diffuse pontine gliomas have a median survival of less than one year and represent a challenge for pediatric oncologists, prompting them to attempt experimental therapies. From 1987 to 2005, 62 children with diffuse pontine glioma, not amenable to curative surgery, were treated according to four successive pilot protocols: (1) concomitant chemo-radiotherapy (etoposide, cytarabine, ifosfamide, cisplatin, and dactinomycin); (2) intensive high-dose courses chemotherapy (cisplatin/etoposide, cyclophosphamide/vincristine/methotrexate) and a subsequent course of myeloablative thiotepa followed by radiation and maintenance chemotherapy; (3) cisplatin/etoposide followed by isotretinoin before, during and after focal irradiation; and (4) iv vinorelbine before, during, and after irradiation. Considering all patients, 77% experienced a transient response to treatment, always detectable after radiotherapy. The progression-free survival (PFS) rate was 25 +/- 6% at one year, median PFS was seven months; overall survival (OS) was 45 +/- 6%, median OS was eleven months: no statistical differences in the four studies in terms of outcome were detected. Despite improved diagnostic, therapeutic, and supportive tools in pediatric neuro-oncology, little has been achieved for patients with diffuse pontine tumors.
Massimino
Maura
M
Pediatric Oncology Unit, Fondazione IRRCCS Istituto Nazionale Tumori, Via Venezian 1, 20133 Milan, Italy. maura.massimino@istitutotumori.mi.it
Spreafico
Filippo
F
Biassoni
Veronica
V
Simonetti
Fabio
F
Riva
Daria
D
Trecate
Giovanna
G
Giombini
Sergio
S
Poggi
Geraldina
G
Pecori
Emilia
E
Pignoli
Emanuele
E
Casanova
Michela
M
Ferrari
Andrea
A
Meazza
Cristina
C
Luksch
Roberto
R
Terenziani
Monica
M
Cefalo
Graziella
G
Podda
Marta
M
Polastri
Daniela
D
Clerici
Carlo A
CA
Fossati-Bellani
Franca
F
Gandola
Lorenza
L
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000401:mortality* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005910:Glioma / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011149:Pons / Q000473:pathology*; D011878:Radiotherapy; D012189:Retrospective Studies