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

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

Fatma E El-Khouly  1   2   3 , Sophie E M Veldhuijzen van Zanten  4   5 , Vicente Santa-Maria Lopez  6 , N Harry Hendrikse  7   8 , Gertjan J L Kaspers  4   5 , G Loizos  9 , David Sumerauer  10 , Karsten Nysom  11 , Kaie Pruunsild  12 , Virve Pentikainen  13 , Halldora K Thorarinsdottir  14 , Giedre Rutkauskiene  15 , Victor Calvagna  16 , Monika Drogosiewicz  17 , Monica Dragomir  18 , Ladislav Deak  19 , Lidija Kitanovski  20 , Andre O von Bueren  21 , Rejin Kebudi  22 , Irene Slavc  23 , Sandra Jacobs  24 , Filip Jadrijevic-Cvrlje  25 , Natacha Entz-Werle  26 , Jacques Grill  27 , Antonis Kattamis  28 , Peter Hauser  29 , Jane Pears  30 , Veronica Biassoni  31 , Maura Massimino  31 , Enrique Lopez Aguilar  32 , Ingrid K Torsvik  33 , Maria Joao Gil-da-Costa  34 , Ella Kumirova  35 , Ofelia Cruz-Martinez  6 , Stefan Holm  36 , Simon Bailey  37 , Tim Hayden  38 , Ulrich W Thomale  39 , Geert O R Janssens  40 , Christof M Kramm  41 , Dannis G van Vuurden  4   5
Affiliations

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

Fatma E El-Khouly et al. J Neurooncol. 2019 Oct.

Abstract

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.

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Conflict of interest statement

The authors declare that they have no conflict of interest.

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