title: Diffuse pontine gliomas in children: changing strategies, changing results? A mono-institutional 20-year experience.
abstract: 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.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRRCCS Istituto Nazionale Tumori, Via Venezian 1, 20133 Milan, Italy. maura.massimino@istitutotumori.mi.it
authors.forename: Maura
authors.initials: M
authors.lastname: Massimino
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Veronica
authors.initials: V
authors.lastname: Biassoni
authors.forename: Fabio
authors.initials: F
authors.lastname: Simonetti
authors.forename: Daria
authors.initials: D
authors.lastname: Riva
authors.forename: Giovanna
authors.initials: G
authors.lastname: Trecate
authors.forename: Sergio
authors.initials: S
authors.lastname: Giombini
authors.forename: Geraldina
authors.initials: G
authors.lastname: Poggi
authors.forename: Emilia
authors.initials: E
authors.lastname: Pecori
authors.forename: Emanuele
authors.initials: E
authors.lastname: Pignoli
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
authors.forename: Cristina
authors.initials: C
authors.lastname: Meazza
authors.forename: Roberto
authors.initials: R
authors.lastname: Luksch
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Graziella
authors.initials: G
authors.lastname: Cefalo
authors.forename: Marta
authors.initials: M
authors.lastname: Podda
authors.forename: Daniela
authors.initials: D
authors.lastname: Polastri
authors.forename: Carlo A
authors.initials: CA
authors.lastname: Clerici
authors.forename: Franca
authors.initials: F
authors.lastname: Fossati-Bellani
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
mesh_terms: 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