Diagnostics and treatment of diffuse intrinsic pontine glioma: where do we stand?

J Neurooncol. 2019 Oct;145(1):177-184. doi: 10.1007/s11060-019-03287-9. Epub 2019 Sep 14.


Introduction: Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines.

Methods: Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively.

Results: Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials.

Conclusion: This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

Keywords: Chemotherapy; Diffuse intrinsic pontine glioma (DIPG); Diffuse midline glioma H3-K27 mutant (DMG K3-27M); Radiotherapy.

MeSH terms

  • Biopsy
  • Brain Stem Neoplasms / diagnosis*
  • Brain Stem Neoplasms / therapy*
  • Combined Modality Therapy
  • Diffuse Intrinsic Pontine Glioma / diagnosis*
  • Diffuse Intrinsic Pontine Glioma / therapy*
  • Disease Progression
  • Humans
  • Prognosis