Diffuse pontine gliomas in children: changing strategies, changing results? A mono-institutional 20-year experience

Autor: Fabio Simonetti, Roberto Luksch, Michela Casanova, Graziella Cefalo, Marta Podda, Geraldina Poggi, Cristina Meazza, Andrea Ferrari, Sergio Giombini, Filippo Spreafico, Carlo Alfredo Clerici, Lorenza Gandola, Giovanna Trecate, Daniela Polastri, Franca Fossati-Bellani, Veronica Biassoni, Monica Terenziani, Maura Massimino, Emilia Pecori, Emanuele Pignoli, Daria Riva
Rok vydání: 2008
Předmět:
Zdroj: Journal of Neuro-Oncology. 87:355-361
ISSN: 1573-7373
0167-594X
Popis: 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.
Databáze: OpenAIRE