Abstract
Background
Patients with diffuse intrinsic pontine glioma (DIPG) have a very poor prognosis. Only radiotherapy (XRT) has proven to be effective in delaying the disease progression. Several chemotherapy schedules have been applied so far, but none demonstrated significant improvements in progression and survival.
Methods
We retrospectively analyzed the clinical data of children diagnosed with DIPG at our center (Pediatric Hospital “Regina Margherita,” Turin, Italy) between 1999 and 2013. Progression-free survival (PFS) and overall survival (OS) were used to describe the outcomes.
Results
Twenty-four children were included in our report. Patients diagnosed before March 2003 (n = 12) were treated with XRT and vincristine (VCR); the remaining 12 patients received XRT and temozolomide (TMZ). Progression-free survival was 18.8 % at 1 year (SE = 7.6 %), while overall survival was 44.1 % at 1 year (SE = 9.9 %). Median PFS was 8.1 months, whereas median OS was 11.2 months. No statistically significant difference in PFS or OS was evidenced between the two treatment groups.
Conclusion
Radiotherapy followed by VCR or TMZ allows obtaining results that are in line with previous reports, with no advantages over other similar treatment schedules.
DIPGs are challenging tumors with a dismal outcome. Further research and newer therapies are urgently needed in order to achieve improvements in survival.
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Acknowledgments
Vallero SG has been funded by The Italian Cord Blood Donors Association (ADISCO).
None of the Authors has financial conflicts of interest. We declare that this manuscript is original, has not been published before, and is not currently being considered for publication elsewhere. The manuscript has been read and approved by all named authors.
The study has been performed in accordance with the 1964 Declaration of Helsinki and its later amendments. All patients, or their parents when needed, gave their informed consent prior to their inclusion in the study.
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Vallero, S.G., Bertin, D., Basso, M.E. et al. Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience. Childs Nerv Syst 30, 1061–1066 (2014). https://doi.org/10.1007/s00381-014-2359-x
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DOI: https://doi.org/10.1007/s00381-014-2359-x