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

Fatma E El-Khouly, Sophie E M Veldhuijzen van Zanten, Vicente Santa-Maria Lopez, N Harry Hendrikse, Gertjan J L Kaspers, G Loizos, David Sumerauer, Karsten Nysom, Kaie Pruunsild, Virve Pentikainen, Halldora K Thorarinsdottir, Giedre Rutkauskiene, Victor Calvagna, Monika Drogosiewicz, Monica Dragomir, Ladislav Deak, Lidija Kitanovski, Andre O von Bueren, Rejin Kebudi, Irene SlavcSandra Jacobs, Filip Jadrijevic-Cvrlje, Natacha Entz-Werle, Jacques Grill, Antonis Kattamis, Peter Hauser, Jane Pears, Veronica Biassoni, Maura Massimino, Enrique Lopez Aguilar, Ingrid K Torsvik, Maria Joao Gil-da-Costa, Ella Kumirova, Ofelia Cruz-Martinez, Stefan Holm, Simon Bailey, Tim Hayden, Ulrich W Thomale, Geert O R Janssens, Christof M Kramm, Dannis G van Vuurden

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

Samenvatting

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.

Originele taal-2Engels
Pagina's (van-tot)177-184
Aantal pagina's8
TijdschriftJournal of Neuro-Oncology
Volume145
Nummer van het tijdschrift1
DOI's
StatusGepubliceerd - okt. 2019

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