Pre-radiation chemotherapy improves survival in pediatric diffuse intrinsic pontine gliomas

Childs Nerv Syst. 2016 Aug;32(8):1415-23. doi: 10.1007/s00381-016-3153-8. Epub 2016 Jul 5.


Background: The median survival of patients with diffuse intrinsic pontine glioma (DIPG) remains less than 1 year. The BSG 98 pre-irradiation chemotherapy protocol showed a significant increase in overall survival. In contrast to current treatment strategies, patients did not have to undergo surgical stereotactic biopsy, which can sometimes lead to complications, to be included in this protocol.

Materials and methods: We retrospectively reviewed all the cases of DIPG that were treated in our department from September 15, 2004 to September 15, 2014. We compared the group of patients who followed our BSG 98 protocol to those who were treated with new targeted therapy protocols where systematic biopsy was required.

Results: Patients in the BSG 98 protocol were treated with BCNU, cisplatin, and methotrexate, followed by radiation at disease progression. Targeted therapy protocols included radiation therapy along with treatment by erlotinib, cilengitide, or an association of nimotuzumab and vinblastine. Sixteen patients were treated with the BSG 98 protocol, and 9 patients were treated with new targeted therapy protocols. Median overall survival was significantly higher in the BSG 98 group compared to the targeted therapy group (16.1 months (95 % CI, 10.4-19.0) vs 8.8 months (95 % CI 1.4-12.3); p = 0.0003). An increase in the median progression-free survival was observed (respectively, 8.6 vs 3.0 months; p = 0.113).

Conclusion: The present study confirms that the BSG 98 protocol is one of the most effective current treatment strategies for DIPG. It may be used as the control arm in randomized trials investigating the use of innovative treatments and may be proposed to families who are averse to biopsy.

Keywords: Chemotherapy; Diffuse intrinsic pontine glioma; Pediatric; Radiotherapy; Stereotactic biopsy; Targeted therapy.

MeSH terms

  • Adolescent
  • Brain Stem Neoplasms / diagnostic imaging
  • Brain Stem Neoplasms / drug therapy*
  • Brain Stem Neoplasms / radiotherapy*
  • Chemotherapy, Adjuvant*
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Glioma / diagnostic imaging
  • Glioma / drug therapy*
  • Glioma / radiotherapy*
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Retrospective Studies
  • Time Factors
  • Treatment Outcome*