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

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

DOI

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  • 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 Slavc
  • Sandra 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
Vis graf over relationer

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.

OriginalsprogEngelsk
TidsskriftJournal of Neuro-Oncology
Vol/bind145
Udgave nummer1
Sider (fra-til)177-184
Antal sider8
ISSN0167-594X
DOI
StatusUdgivet - 2019

ID: 59001643